Usmle 01 1415 Manual CD

Cardiology
American Manual
of Examination
in Medicine
(2CK)
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Cardiology
American Manual
of Examination
in Medicine
(2CK)
Authors
Rafael Salguero
Alfonso Jurado
Roberto Martn
David Filgueiras
Javier de Juan
Grard Loughlin
Sem Briongos
Felipe Dez
A m e r i c an Ma nua l o f Ex am i na ti o n i n Me dicine (2CK)
Index
01. Physical Examination........................................................................ 1
1.1.
1.2.
1.3.
1.4.
Cardiac Auscultation ........................................................................................................ 1

Murmurs ............................................................................................................................................. 2
Arterial Pulse ................................................................................................................................ 2
Jugular Venous Pressure ........................................................................................... 3
02. Electrocardiogram................................................................................. 4
2.1.
2.2.
2.3.
2.4.
2.5.
Cardiac Frequency ............................................................................................................... 4

Rhythm ................................................................................................................................................. 4
Electrical Axis............................................................................................................................... 4
Intervals ............................................................................................................................................... 4
Main ECG Anomalies ........................................................................................................ 5
05. Cardiomyopathies ............................................................................. 27

5.1.
5.2.
5.3.
5.4.
Dilated Cardiomyopathy ........................................................................................ 28

Hypertrophic Cardiomyopathy .................................................................... 29
Restrictive Cardiomyopathy.............................................................................. 32
Other Cardiomyopathies ....................................................................................... 33
06. Coronary Artery Disease ......................................................... 35

6.1. Concepts ........................................................................................................................................ 35
6.2. Angina Pectoris .................................................................................................................... 37
07. Acute Coronary Syndrome .................................................. 40
3.1. Bradycardia..................................................................................................................................... 6
3.2. Tachycardia..................................................................................................................................... 8
7.1. Acute Coronary Syndrome without

ST segment elevation ................................................................................................. 40
7.2. Acute Coronary Syndrome with
ST segment elevation ................................................................................................. 42
7.3. Complications of myocardial infarction .......................................... 45
04. Heart Failure ............................................................................................... 17
08. Dyslipidemia .............................................................................................. 48
03. Arrhythmias .................................................................................................... 6
4.1.
4.2.
4.3.
4.4.
Classification ............................................................................................................................ 18
Pathophysiology and Clinical Characteristics ......................... 18
Complementary Tests ................................................................................................. 19
Treatment of Heart Failure................................................................................... 20
09. Hypertension............................................................................................. 49
9.1. Essential Hypertension ............................................................................................. 49
9.2. Secondary Hypertension ....................................................................................... 52
9.3. Special Cases ............................................................................................................................ 52
Card iology
10. Pericardial Disease............................................................................ 53 13. Syncope ........................................................................................................... 75

10.1. Acute Pericarditis............................................................................................................... 53
10.2. Pericardial Tamponade ............................................................................................ 56
11. Valvulopathies ......................................................................................... 58

11.1.
11.2.
11.3.
11.4.
11.5.
11.6.
Basic Concepts ...................................................................................................................... 58

Mitral Stenosis........................................................................................................................ 61
Mitral Insufficiency.......................................................................................................... 62
Aortic Stenosis ....................................................................................................................... 64
Aortic Insufficiency ......................................................................................................... 66
Right Heart Valve Disease ..................................................................................... 67
12. Vascular Disease .................................................................................. 67

12.1.
12.2.
12.3.
12.4.
12.5.
Aortic Aneurysm................................................................................................................. 67
Acute Aortic Syndrome ............................................................................................ 68
Deep Vein Thrombosis............................................................................................... 70
Peripheral Artery Disease...................................................................................... 72
Lymphedema .......................................................................................................................... 75
13.1. Differential Diagnosis.................................................................................................. 75
14. Miscellaneous .......................................................................................... 77

14.1.
14.2.
14.3.
14.4.
14.5.
14.6.
14.7.
14.8.
Shock ................................................................................................................................................... 77
Rheumatic Fever ................................................................................................................. 78
Myocarditis ................................................................................................................................. 78
HIV-Related Cardiomyopathy ......................................................................... 79
Bacterial and Parasitic Myocarditis.......................................................... 79
Giant Cell Myocarditis................................................................................................. 80
Radiation Myocarditis ................................................................................................. 80
Cardiac Tumors ..................................................................................................................... 81
Card iology
Physical
Chapter 01
of the apex instead of an apical impulse is detected, constrictive

pericarditis or hypertrophy and RV displacing behind the LV must
be suspected.
Examination
Some cardiac diseases present characteristic features when observed

(Table 1).
ILLNESS
CARDIAC INVOLVEMENT
1.1. Cardiac Auscultation
OBSERVATION DATA
Sleepapneahypopnea
syndrome(SAHS)
Cor pulmonale
Atrial fibrillation
Bradycardia and tachycardia
Obesity
Short neck
Daytime sleepiness
Duchenne dystrophy
Posterobasal left ventricular

dystrophy
Lower limbs pseudohypertrophy

Lumbar lordosis
Gowers sign
Steinert myotonic
dystrophy syndrome
Infra-Hisian AV block
Ventricular tachycardia
Inexpressive myopathic facies

Frontal baldness
Cataracts
Kearn-Sayre
syndrome
Infra-Hisian AV block
Ophtalmoplegia
Unilateral ptosis
Pigmentary retinopathy
Marfans syndrome
Ascending aneurysm
Aortic insufficiency
Mitral prolapse
Ehlers-Danlos
syndrome
Aneurysm or aortic dissection
Skin and joint hypermobility

Detached retina
Rendu-Weber-Osler
disease
AV pulmonary fistulas
Hemorrhagic telangiectasia in mucous

membranes
Carcinoid syndrome
Pulmonary or tricuspid
valvulopathy
Flushing and edematous episodic

associated with hypotension
Sarcoidosis
AV block
Cor pulmonale by
pulmonary involvement
Lachrymal glands hypertrophy
Very tall. Enlarged limbs

Aracnodactilia
Upward lens dislocation
Pectum excavatum/carinatum
Hypercholesterolemia Coronary artery disease
Xanthelasma
Corneal arcus
Tendon xanthomas
Homocystinuria
Downward dislocation of the lens
Systemic, pulmonary or
coronary thrombosis
Infective endocarditis Endocarditis
The first heart sound (S1) is produced by

the closing of the valves (mitral valve, more
powerful, and tricuspid valve). The second
(S2) is produced by the closing of semilunar
valves (aortic, more powerful, and pulmonary, in this order).
Rhythms producing continuous changes in
PR interval (complete AV block) or in RR
interval (such as atrial fibrillation) cause a
variable intensity of S1. A small S1 splitting
in inspiration is a normal finding, more noticeable at times. This splitting is increased
in right bundle branch block. However,
it is diminished or reversed in left bundle
branch block, mitral stenosis or left atrial
myxoma.
S2 splitting during inspiration is normal and
generally greater than S1 splitting, since at
this stage in breathing, negative pressure
increases pulmonary venous capacitance
(the lung may store much more blood) delaying closure of the pulmonary valve. S2
splitting is greater when there is a delay
in RV ejection, as in right bundle branch
block; in pulmonary stenosis or in atrial
or ventricular septal defects with no pulmonary hypertension, or when the aortic
valve closes slightly before the pulmonary
valve.
Roth spots in retina

Osler nodes
Petechiae in mucous membrane
A wide and fixed splitting of the second

heart sound (S2), (not increased during
inspiration), is characteristic of ostium
Acute rheumatic
Rheumatic carditis
Subcutaneous nodes in elbows, malleolus,
secundum atrial septal defect (ASD). Parafever
and back of hands, feet or vertebra
doxic splitting of S2 occurs when aortic
Table 1. Characteristic features of some diseases with cardiovascular manifestations
valve closure is delayed (heard best during expiration, being sometimes the only
Palpation: point of maximal impact must be examined in the left lateral decu- S2 during inspiration). This phenomenon can be found in left bundle
bitus position, better detected in forced expiration, and localized in a 2-3 cm branch block (LBBB), premature ventricular beats, aortic stenosis, or
diameter area normally located in the 5th intercostal space at the midclavicu- in the presence of RV shorter ejection time, as in pulmonary hypertension with RV failure.
lar line. A heart impulse, soft and short, or unique movement will be felt.
In concentric left ventricular hypertrophy, the point of maximal impact
may be larger (more than 3 cm) and continuous during systole. In LV
dilatation, the point of maximal impact displaces down and left. LV aneurysms may cause another ectopic focus (localization of aneurysm).
In hypertrophic obstructive cardiomyopathy, a systolic double
point of maximal impact may be felt. When a systolic retraction
The third heart sound (S3) is produced by rapidly rushing blood flow
entering either the right or left ventricle, performing auscultation
over the apical impulse of each ventricle. S3 may be physiologic in
children or in young people (especially women) and in situations involving high cardiac output (fever, pregnancy). In adults, this may be
an indicator of disease (systolic dysfunction, ventricular dilatation,
serious ventricle-atrial regurgitation).
The fourth heart sound (S4) corresponds to an atrial contraction generated by sudden deceleration of the blood entering the ventricle with
decreased compliance (associated with hypertrophy because of high
blood pressure, aortic stenosis, hypertrophic cardiomyopathy, mitral
insuciency, ischemia, advanced systo-diastolic dysfunction, hyperdynamia). It is produced as a late diastolic sound but it does not occur
when atrial fibrillation exists. As S3, it is a low-frequency sound best
audible with the bell of the stethoscope in the apical zone of the affected ventricle.
1.2. Murmurs
ture, anemia or pregnancy, it is frequent to auscultate a mild mesosystolic

normal murmur due to blood turbulence when rapidly entering the aorta
or pulmonary artery. Stills murmur, very frequent in children, is also reported. It appears from birth to adolescence and is heard in the pulmonic
area. It is thought to be produced by vibration of the pulmonary valves. In
the elderly, aortic sclerosis innocent murmur is very frequent (without
stenosis) by degenerative fibrosis of aortic leaflets, becoming more rigid
and increasing vibration when blood flows through. Gallavardin phenomenon is characteristic in this murmur and has a harsh noisy sound heard
in the aortic focus (due to blood turbulence in the ascending aorta) and a
musical sound in cardiac apex.
There are some maneuvers altering the intensity or features of murmurs and helping determine their nature (Table 2):
Aortic focus
EFFECTS ON MURMUR
Pulmonary focus
MANEUVER
Accessory aortic
focus
P
Aa
Increase
Decrease
HOCM and MVP
Most murmurs
Squatting
Most murmurs
HOCM and MVP
Amyle nitrite,
vasodilatation
Aortic stenosis, HOCM, Mitral and aortic

MVP
regurgitation
Handgrip,
vasoconstriction
Mitral and aortic

regurgitation
S4
Valsalva and
bipedestation
Aortic stenosis, HOCM,

MVP
Table 2. Maneuvers helping determine the nature of murmurs
1.3. Arterial Pulse

Mitral focus
Tricuspid focus
The wave of normal arterial pulse has a rapid climb (commonly called
percussion wave), with a detectable anacrotic notch in invasive records
of central pulses, though nonpalpable, reaching a unique well defined
Figure 1. Auscultation focuses

Murmurs are produced by turbulent flow
through valves due to organic diseases or to
situations such as hyperflux in a valve or blood
vessel.
Auscultation focuses are those in which murmurs are heard more clearly, determined by
transmission and acoustic impedance of the
thorax (Figure 1). Furthermore, there are characteristic radiation patterns (i.e: aortic stenosis
murmur radiates to carotid arteries and supraclavicular areas. In mitral insuciency, when
the regurgitant jet is directed posterolaterally,
the murmur radiates to the axilla and the left
scapula.). In valvulopathy chapter, the characteristics of murmurs will be defined.
According to the stage in which they appear,
murmurs may be systolic, diastolic or continuous (systo-diastolic). Innocent murmurs (with no
significant organic disease) are mesosystolic. So,
in situations of hyperdinamia such as tempera-
ARTERIAL PULSE
ETIOLOGY
CHARACTERISTICS
Celer et magnus
or hyperkinetic
Increase in cardiac output volume,

Strong and short heartbeat
decrease in peripheral resistance (aortic Wide differential pressure
insufficiency, anemia, fever)
Hypokinetic
Hypovolemia, LV insufficiency (AMI,

mitral stenosis)
Bisferiens
Aortic insufficiency, HOCM
Two systolic peaks
Dicrotic pulse
Dilated cardiomyopathy in low

cardiac output
Generally associated with alternating
pulse
Two peaks: one, systolic and the

other, protodiastolic
Parvus et tardus
Decrease in cardiac output volume

(aortic stenosis)
Flat (weak) and prolonged wave

Reduced differential pressure
Alternating
In ventricular decompensation, with S3

and S4
Pulse amplitude variation
Bigeminus
Ventricular premature contraction,

digitalis intoxication
Alternation of normal and

extrasystole heartbeats
Paradoxic
Cardiac tamponade, respiratory

tract obstruction, poor blood return
(sometimes, in constrictive pericarditis)
More than 10 mmHg drop in

systolic pressure during inspiration
Amplitude-diminished heartbeat,
possible tachycardia
Table 3. Some abnormalities of arterial pulse
Card iology
rounded peak (called pre-dicrotic wave), followed by a slower decline,

interrupted by a notch or dicrotic notch (palpable at times) caused by the
closure of the aortic valve, after which a short climb (dicrotic wave) is followed by a continuous decrease till the next beat. With aging and arterial
sclerosis, the initial wave tends to be more abrupt reaching a higher peak.
The main anomalies of amplitude or waveform are described in Table 3.
1.4. Jugular Venous Pressure

External jugular vein is a static blood column (nonpulsating) whose
maxim height contributes to estimating the right atrium midpoint pressure, measured in centimeters of elevation above the sternal angle of
Louis (manubrio sternal joint, which lies about 5 cm above the RA)
The external jugular vein is frequently located immediately over the
clavicle, the subject being elevated at 30, which corresponds to 3 cm
above the sternal angle (which equates to 8-9 cm H2O) (Figure 2).
Central venous pressure may also be measured by estimating the height

over the sternal angle where a and v waves of internal jugular pressure are appreciated. The most frequent cause of elevation of vein pressure is elevated ventricular diastolic pressure.
Hepatojugular reflux (more correctly named abdominojugular reflux) is
tested by firmly pressing for 10-15 seconds over the center of the abdomen of the patient breathing normally so that they do not perform a
Valsalva maneuver. In healthy subjects, there is no evident elevation or
it is mild when compressing, whereas the test is considered to be positive if JVP visibly elevates during compression, sustained for 10-15 seconds and falls more than 4 cm when pressure is released. This positivity
denotes hypofunction of the right ventricle.
Kussmauls sign consists of an increase in JVP on inspiration (which diminishes in physiologic conditions, since blood accumulated in jugular
veins is backed up into the RA due to negative pressure in thorax during inspiration). It may appear in any severe right diastolic heart failure
since excessive blood volume reaching the right cavities on inspiration
produces an increased pressure, which is characteristic of constrictive
pericarditis, restrictive cardiomyopathy and RV infarction.
Normal JVP has two positive waves (Figure 3), a wave (presystolic
wave) represents elevated pressure caused by right atrial contraction
and occurs after diastolic filling of the ventricle shortly before the first
heart sound.
Louis angle
5 cm
a wave
v wave
Right atrium
y depression
x depression
45
1R
2R
Figure 2. Central venous pressure measurement (CVP)

Diastole
However, external jugular distension does not always indicate elevation

in atrium pressure since a vein valve may, at times, distend the external
jugular vein even at low pressures.
ABSENT
Atrial fibrillation
DIMINISHED
Dilated RA
Atrial fibrillation Dilated RV

Severe tricuspid
regurgitation
Cardiac
tamponade
Tricuspid
obstruction
Tricuspid stenosis
Pulmonary stenosis
Pulmonary
hypertension
Pericardial tamponade (constrictive

pericarditis)
Tricuspid regurgitation
Intra-atrial communication
Tricuspid
Constrictive pericarditis
stenosis
Severe right cardiac failure
RA myxoma Severe tricuspid regurgitation
Table 4. Jugular vein pulse waves
Diastole
Figure 3. Jugular venous pressure
INCREASED
Hypertrophic
RA
Systole
The descent x wave initiates by atrium relaxation and is intensified by the bulging of the
tricuspid valve into the right atrium, at the beginning of ventricular contraction.
v wave is due to the filling of RA during ventricular contraction (closed tricuspid).
Upon start of ventricular diastole and opening
of the tricuspid valve, blood passes suddenly
from RA to RV, appearing y descent (diastolic collapse), which is less eminent and deep
than x descent.
The main anomalies of JVP are shown in Table 4.
Electrocardiogram
2.3. Electrical Axis

The electrical axis of any wave in ECG relies on the concept of a vector
which describes the motion in the frontal plane (expressed in degrees)
of such depolarization wave. Normal P, QRS and T aim at the lower-right
quadrant of the heart.
Chapter 02
Standard surface electrocardiogram (ECG) has 12 vector views or leads,

6 of which are limb leads (I, II, III, aVR, aVL, aVF) and the other 6 are
precordial leads (V1 to V6). Each one represents cardiac electrical activity from dierent perspectives (Figure 4).
V9
The normal QRS axis is between -30 and +150. An axis beyond -30
is a left-deviated axis (as in left anterior hemiblock [LAH]), whereas a
right heart axis (beyond +150) is deviated to the right (typical in left
posterior hemiblock [LPH]).
V8
V7
aVR 150
V6
aVL 30
V5
DI 0
aVF 90
DII 60
2.4. Intervals
V4
V3
V4R
DIII 120
A quick way to measure QRS axis is by searching the lead in the frontal
plane in which QRS is the most positive (highest). The axis has to be
situated near such a lead.
V3R
V1
V2
In normal ECG, the paper moves forward 25 mm/sec. This is why 1 mm

equals 40 ms. In Figure 5, normal intervals are shown.
Remember that QT interval depends on cardiac frequency: the faster
the heart rate, the shorter the interval. There exist many forms to normalize or correct the absolute QT value: the normal QTc value is that
below 440 ms (450 in women).
Figure 4. Surface ECG standard leads

It is highly recommended to follow a systemic order when analyzing
electrocardiographic records.
There are several correction formulas. Bazett s is the most commonly

used formula: Corrected QT = measured QT/ RR
(RR represents the time, in seconds, from the onset of one QRS complex
to the onset of the next QRS complex, whose QT is being measured).
2.1. Cardiac Frequency

In normal resting, CF ranges from 60-100 bpm, and less in athletic
young individuals. Figures below 60 bpm constitute bradycardia, and
above 100 bpm, tachycardia.
2.2. Rhythm
3 mm
3-5 mm
< 3 mm
< 440 ms
It is necessary to dierentiate p waves (atrial activation) from QRS

complexes (ventricular activation).
It must be defined whether ventricular rhythm (intervals between
QRS complexes) is rhythmic or not (extrasystole, atrial fibrillation) and
whether QRS complexes are preceded by a p wave or not (in which
case a nodal or ventricular nature of the rhythm is denoted).
Greater p waves number than QRS complexes indicate some block
in nodal conduction (whether physiologic or pathologic), whereas greater QRS complexes number indicate nodal or ventricular
rhythm.
QT
QT
Figure 5. Normal timing in surface ECG
Card iology
2.5. Main ECG Anomalies

P wave (better recorded in DII and V1). In RA hypertrophy, P wave
produces a tall, peaked P in its first part (P pulmonale) in DII, increasing
its first positive component in V1. LA hypertrophy produces changes in
the second part of the wave, with a wide and uneven P in DII (P mitral),
and biphasic prevailing the 2.nd negative component in V1.
PR Interval. It enlarges in AV blocks, being sustained in 1.st Degree
blocks with a progressive enlargement in type I 2.nd Degree blocks
(Wenckebach) and variable in AV dissociation (as complete 3rd Degree AV block). It shortens in ventricular pre-excitation syndromes
(Wol-Parkinson-White).
QRS complex. A Broad QRS complex (QRS > 120 ms) may be due to
aberrant ventricular depolarization in which impulses are not triggered simultaneously in the His-Purkinje system; this occurs when
a His bundle branch block is present, when ventricular pre-excitation is produced by an accessory pathway with anterograde conduction, under the influence of type I anti-arrhythmic drugs which
slow down the conduction, in hyperkalemia or when the impulse

has a ventricular origin (ventricular extrasystole, ventricular tachycardia, infra-Hisian rhythm in complete atrioventricular block, AIVR
or pacemaker-induced ventricular rhythm).
A bundle branch block is considered to be complete if QRS is longer
than 120 ms, or incomplete if its length is less than 120 ms. QRS specific morphology patterns enable dierentiation of right bundle branch
block (RBBB) (rSR in V1-V2, wide s wave in DI and V5-V6) from left
bundle branch block (LBBB) (QRS is mainly negative in V1 as are rS or QS
and RR in V5-V6). In bundle branch block the repolarization sequence
is also aected (Figure 6).
Normal Q wave -a small physiologic wave appreciated in leads I, II, III,
aVF, aVL and V5-V6 indicates depolarization of interventricular septum.
When Q wave is broader than 40 ms and deeper than 2 mV (or 25%
of depth of QRS complex), it is considered a pathologic Q, which usually indicates transmural infarction located in the area explored by the
lead in which the pathologic Q wave appears. Sometimes, pathologic
Q waves are appreciated without infarction
in hypertrophic cardiomyopathy or in WolParkinson-White syndrome.
RV hypertrophy moves the ventricular depolarization vector right and front, as a consequence a QRS right axis deviation appears (>
90). In precordial leads, as for the turning
of direction the vector points at V1 and V2
leads, instead of a QRS complex dominantly
negative, QRS is dominantly positive with a
dominant R wave taller than S wave depth,
along with aberrant secondary repolarization
(T-wave inversion and ST depression).
This is the traditional right ventricular hypertrophy or type A growth. There exists a right
ventricular hypertrophy type C, characteristic of cor pulmonale, in which hypertrophy
of the right ventricle outflow tract causes a
nondiscernible axis (isodiphasic complexes in
all frontal leads). Acute right ventricular volume overload (i.e. in pulmonary embolism)
may cause these alterations and the characteristic SIQIIITIII pattern (S in DI, Q and
negative T in DIII).
Figure 6. Left bundle branch block (top) and Right bundle branch block characteristics
in precordial leads
LV hypertrophy does not modify the QRS axis

since it is, by itself, deviated to the left. However, it will produce high voltages in QRS, with
aberrancies in repolarization (inversion of T
wave and ST depression, especially in I, aVL,
V5-V6 leads). There are several ECG criteria
to determine the existence of LV hypertrophy, although the existing dierent measure
indexes generally show low sensibility but
better specificity (Sokolov-Lyons, Cornell).
This is why, at present, echocardiography is
the first-choice test to prove hypertrophy
(even though cardiac magnetic resonance
may be more precise; Figure 7).
in progression of acute pericarditis, in apical hypertrophic cardiomyopathy (giant negative T waves), and in V1-V3 leads in RV arrhythmogenic cardiomyopathy. Negative T waves may be normal in
V1-V3 leads, especially in women and children (child pattern) and in
DIII lead. Generally, T wave is negative in aVR.
Occasionally, flat T waves are detected, defined as unspecific alteration of repolarization, and they are usually a normal variant
(especially frequent following cardiac surgery), though it is recommended to rule out subjacent ischemia. Anxiety, physical exercising (including ergometry), hyperventilation, sustained tachycardia,
postprandial period, orthostatism, acute pancreatitis (aecting DII,
DIII and aVF), and acute CVA may flatten or even invert T waves.
Figure 7. ECG obtained from a patient suering biventricular dilated
cardiomiopathy. Hypertrophic changes are remarkable as RBBB and
repolarization abnormalities
ST segment. Elevated ST to values over 1 mm may indicate transmural

lesion current (convex upwards), ventricular aneurysm (if it persists
after acute myocardial infarction [AMI]), pericarditis (diuse upwards
ST elevation), Brugada syndrome (elevation in V1-V3 with negative
T and incomplete right bundle branch block) or often, benign early
repolarization (normal variant seen in young people with dominant
vagal hypertonia, with slight convex ST elevation and notching of the
J-point. This anomaly has been considered of no clinical relevance for
a long time, but recently, it has been more frequently reported in patients with sudden cardiac death than in the general population).
ST depression may also appear during ischemia episodes in stable/
unstable angina or subendocardial acute myocardial infarction. It may
also indicate ventricular overload (secondary to hypertrophy, generally in a descending slope), digoxin eect or bundle branch block.
T wave. It elevates with subendocardial ischemia, in hyperkalemia
(peaked T), in the rare congenital short QT syndrome, upon LV volume overload (dilated cardiomyopathy) or in some cases of hypertrophic cardiomyopathy. T wave becomes negative in the presence
of transmural ischemia (symmetrical and deep), as a consequence
of infarction accompanying Q wave, in ventricular pre-excitation,
upon ventricular overload (in ventricular hypertrophy or dilation),
DISEASE
Sinus nodal
dysfunction
ECG MANIFESTATIONS
There may exist:
Bradycardia (< 55 bpm)
Bradycardiatachycardia
Corrected QT interval shortens in hypercalcemia and, occasionally, under digoxin treatment, as well as in congenital short QT
syndrome. It prolongs in hypocalcemia, in hypokalemia and other
endocrine metabolic alterations, and in acute ischemia because of
the administration of drugs prolonging QT interval (class IA and III
anti-arrhythmic drugs, macrolide and quinolone antibiotics, tricyclic
antidepressants) or in congenital long QT syndrome. Negative or inverted U wave may indicate underlying ischemia, although it is not
a frequent finding.
Arrhythmias
Chapter 03
3.1. Bradycardia (Table 5)

Underlying mechanisms in bradyarrhythmias may be related to an alteration in the impulse generation system (as in sick sinus syndrome, Figure 8), or to an alteration in the impulse conduction system. This forces
a pacemaker slower than sinus pacemakers to set the heart rhythm (the
TREATMENT
Pacemaker in (DDD/R):
Symptoms
Symptomatic daytime
pauses > 3 s
A-V CONDUCTION ALTERATIONS

(Pacemaker of choice DDD/R. In AF, the indication is VVIR)
First-degree block
PR > 0.20 sec (pacemaker indication DDD/R. In

AF, the indication is VVIR)
Consider pacemaker
according to PR > 0.3 s
and symptoms
ASSOCIATION
Second-degree
block (Mobitz I)
Repeating cycles with progressive PR increase

Pacemaker if symptomatic
until a P wave block, and then restarting the cycle
Inferior AMI. Drugs
AV node
Second-degree
block (Mobitz II)
Failure in the conduction of some impulses

(P not followed by QRS)
Anterior AMI
His bundle
Third-degree block A-V Dissociation
Pacemaker
Pacemaker
Table 5. Characteristics and treatment of bradyarrhythmias
LOCATION
Card iology
Figure 8. Lead aVF ECG revealing characteristic signs of sinus dysfunction with signicant sinus bradycardia
more distal the block point the more severe it usually is (Figure 9 and
Table 6).
whether infra-Hisian or supra-Hisian, as well as the sinus node dysfunction. If electrophysiologic study results are inconclusive and, mainly, if
syncope recurrence is very sporadic, a subcutaneously implanted Holter
may be useful.
Figure 9. Influence of complete AV block point in escape rhythm
THIRD DEGREE
AV BLOCK
NODAL
(SUPRA-HISIAN)
INFRANODAL
(INFRA-HISIAN)
Escape rhythm
40-60 bpm
20-40 bpm
Response to atropine
QRS
Normal (< 0.12)
Wide (> 0.12)
Prognosis
Good
Poor
Table 6. Complete AV block

The most frequent reversible cause is drugs which slow conduction. The
most frequent irreversible cause is idiopathic degenerative fibrosis of
the conduction system (Figures 10 and 11).
Figure 11. Second-degree AV block. Mobitz I (A) and Mobitz II (B)
SITUATION
INDICATION
Biphasic or triphasic block

Sinus dysfunction
First degree AV block
Second degree Type I AV block
Only in case of symptoms

secondary to bradycardia
(asthenia, syncope, or repetitive
presyncope)
Occasionally, to treat patients
with blocker drugs
Second degree Type II AV block

High degree AV block
Third degree AV block
Alternating bundle branch
block
ALWAYS
Other situations
Figure 10. First-degree AV block
Carotid sinus hypersensitivity

Pure cardioinhibitory neurally
mediated syncope
Ventricular arrhythmias
because of bradycardia
Cardiac resynchronization
therapy
Medical treatment usefulness is limited in acute transient management; permanent pacemaker implantation is the required solution in
nonreversible cases (Table 7).
Table 7. General indications of pacemakers
Whenever in doubt of indication of permanent pacemaker implantation

(patients with syncope and bundle branch block or bifascicular block,
patients with 2:1 AV conduction ratio), an electrophysiologic evaluation
may be performed in order to evaluate the level of conduction disease,
Ventricular stimulation with pacemaker from the RV apex produces a

QRS similar to that in left bundle branch block (Figure 12). Biventricular
stimulation in patients with resynchronization usually reaches a narrower fusion QRS than patients basal QRS.
There are multiple causes, since in most cases it is the result of stress, anxiety, fever, anemia, hypovolemia, hypotension, exercise, thyrotoxicosis, hypoxemia, heart failure, pulmonary embolism, adrenergic stimulus. Thus, sinus tachycardia is, in most cases, a sign that should alert to the possibility of
the patient presenting a potentially severe illness. Therefore, the root cause
should be treated. Rare inappropriate sinus tachycardia, common among
female health personnel, does not have a recognizable triggering factor, and
may require the use of -blockers, calcium antagonists or ivabradine.
Figure 12. 12-lead ECG from a patient in sustained atrial brillation with
VVIR pacemaker implantation stimulating RV apex at 100 bpm
Chronic right apical pacing is harmful to contractile function in patients

with systolic dysfunction. In these cases, biventricular stimulation must
be considered (resynchronization).
3.2. Tachycardia
Figure 13. 12-lead ECG of patient with sinus tachycardia and no

structural heart disease
Premature Beats (Table 8)

ATRIAL
PREMATURE BEATS
ECG
alterations
Premature P wave
Normal QRS
Subsequent
Noncompensatory
pause (usually)
Atrial Fibrillation
VENTRICULAR
PREMATURE BEATS
Premature QRS
Wide QRS
Compensatory
Prevalence
Present in 60% of adults
Consequences
Usually few
Treatment
Remove excitants Remove excitants

-blockers if discomfort
-blockers if
discomfort
in patients with no heart
Consider ablation
disease
in special cases
Special emphasis on
treatment of underlying
heart disease, if there is one,
with -blockers
In special cases
(tachycardiomyopathy
induced by frequent
premature beats),
radiofrequency ablation
Few in the absence of heart

disease
Clinical importance,
especially in ischemic heart
disease, if they are frequent,
complex or with R-on-T
phenomenon
Table 8. Types of premature beats
Sinus Tachycardia
This is sinus rhythm with a rate of over 100 bpm, with P waves identical
to the sinus (Figure 13). This arrhythmia starts and finishes gradually,
and its frequency may be slowed temporarily with vagal maneuvers.
Atrial fibrillation (AF) is the most common type of arrhythmia after premature beats and also the most frequent sustained chronic arrhythmia. ECG reveals disorganized atrial activity without P waves, which
are replaced with irregular waves of the T-QRS complex (f waves, at
350-600 bpm) showing variable and irregular (complete arrhythmia)
ventricular conduction (ventricular response; Figure 14).
Figure 14. Atrial brillation

AF is classified into first episode (first documented AF episode of the
patient, regardless of duration), paroxysmal (episodes of spontaneous
cardioversion during the first week, usually within first 24-48 hours),
persistent (it does not cardiovert spontaneously within seven days, although it may be attempted) or permanent (chronic, it does not cardiovert spontaneously nor is it pursued). Just as any other arrhythmia, it
is called recurrent when there is more than one episode and sustained
when it lasts for more than 30 seconds.
AF may appear both in people with structural heart disease and in
healthy people. Among its causes, the following are found: emotional
stress, post-surgery, acute alcohol intoxication, hypoxemia, fever, hypercapnia, metabolic or hemodynamic alterations, hyperthyroidism,
Card iology
POINTS
CHA2DS2-VASC
SCORE
C (congestive heart failure or ventricular dysfunction with EF < 40%)
H (hypertension)
1.3
A2 (age > 75)
2.2
D (diabetes mellitus)
3.2
S2 (prior stroke or TIA or thromboembolism)
4.0
V (coronary, aortic or peripheral vascular disease)
6.7
A (age 65-74)
9.8
Sc (female gender)
9.6
FACTOR
ANNUAL ADJUSTED
STROKE RISK RATE (%)
6.7
15.2
Table 9. Stroke risk calculation in atrial brillation according to score system CHA2DS2-VASc
valvulopathies, hypertensive heart disease, almost any structural heart
disease, chronic obstructive pulmonary disease, sleep apnea, constituting a part of the bradycardia-tachycardia syndrome.
AF may range from asymptomatic to result in hemodynamic intolerance
with syncope or acute pulmonary edema. Arrhythmia management involves assuming that its presence indicates a steep increase in stroke
risks, especially (although not exclusively) caused by the formation of
clots in the left atrial appendage that once detached are responsible
for strokes. It is thus necessary to assess in all cases the need for anticoagulant treatment, which will be necessary for almost every patient
with heart disease or underlying prosthetic cardiac valves, as well as
when suggested according to the CHADS-VASc score of calculated risk
(Table 9).
Currently, there are two potential treatment strategies for atrial fibrillation:
Rate control strategy. It does not aim to restore sinus rhythm but to mitigate potential complications which may result from a permanent AF.
Rhythm control strategy. Aimed at restoring sinus rhythm and sustaining it for the longest possible time.
Various clinical trials have shown that neither strategy is better than the
other in terms of mortality. Hence, a strategy will be selected based on
patient characteristics and arrhythmia tolerance.
The figure shows general management of a patient with atrial fibrillation (Figure 15, next page).
Atrial Flutter
The common typical flutter in an atrial tachycardia caused by a macroreentry around the tricuspid annulus, which cycles at 250-350 bpm
(in a counter-clockwise direction in the common type, clockwise in the
reverse type) and from which atrial depolarization takes place, showing in the ECG saw-toothed regular atrial activity (F waves, positive
in V1 and negative in inferior leads) with scarce mechanical activity
(Figures 16 and 17).
Common flutter
V1
aVF
Atypical flutter
V1
aVF
Figure 16. Anatomic reconstruction with nonuoroscopic navigation

system of atrium and right atrial appendage (blue), cava veins (brown)
and coronary sinus (purple). A diagnostic catheter can be seen around the
tricuspid ring, as well as radiofrequency lesions (orange spots) performed
on the cavotricuspid isthmus for common utter circuit ablation
Figure 17. ECG of common utter and atypical utter in leads V1 and aVF
Figure 15. Clinical management of atrial brillation

Common flutter is very frequent among patients with COPD or sleep
apnea. There are atypical flutter circuits less frequently around other
obstacles (foramen ovale, cava veins, post-surgery scars; Figure 18).
The most eective treatment is synchronized electrical cardioversion,
which usually requires low energy. Anti-arrhythmic drugs are very ineective, except dibutilide and dofetilide, which shows only moderate
ecacy.
Although stroke risk seems somewhat lower, anticoagulation treatment must be conducted as analogous to AF. AV node slowers are
less eective than in AF to control ventricular response.
For recurrence prevention, drugs are also very limited, thus with
recurring common or reverse flutters, or in a poorly tolerated first
episode, the procedure of choice is radiofrequency ablation of the
cavotricuspid isthmus (part of the reentry circuit), with a success
10
rate of over 90% in common flutter. In atypical cases, ablation efficacy is lower.
Atrioventricular Nodal Reentrant

Tachycardia (AVNRT)
It is the most common paroxysmal supraventricular tachycardia (Figure 19).
In atrioventricular reentrant tachycardia (AVNRT), it is speculated that a
double pathway between the atrium and AV node exists (although this
phenomenon is also present in subjects with no tachycardia), a slow
pathway (a, with short refractory period) and a fast pathway (b, with
long refractory period), which may establish a reentrant circuit between
them (Figure 20).
Card iology
Figure 18. Common and atypical utter circuits: atrial layout representing the placement of atrial macroreentry in common utter
and atypical utter because of atriotomy scar
portions (pseudo S in inferior leads or pseudo R in V1; Figure 21).

The close match between atrial and ventricular
contractions causes cannon a-waves in the
jugular venous pulse, regular in all tachycardia
beats (frogs sign), and the patient frequently refers neck palpitations.
Electrical cardioversion is the treatment of
choice, if there is severe hemodynamic compromise. If there is no compromise, maneuvers are adopted to block the AV node to interrupt the tachycardia: vagal maneuvers (carotid
sinus massage), intravenous infusion of drugs
such as adenosine or verapamil. It is best to
conduct the treatment under electrocardiographic monitoring, since the use of adenosine
may cause atrial fibrillation (less than 10%).
Figure 19. Paroxysmal supraventricular tachycardia (AV nodal reentrant tachycardia)
Figure 20. Lewis diagram showing the start of common AV nodal reentrant tachycardia
Tachycardia starts and finishes abruptly, and may cause palpitations, hypotension, syncope. It is prevalent among middle-aged women.
ECG outside of crisis periods is normal, and during tachycardia it shows
a retrograde P wave almost simultaneous to QRS thus altering their final
For prophylaxis of new episodes, drugs of

choice are those slowers of the fast pathway
of the AV node, such as -blockers, verapamil
or diltiazem. Anti-arrhythmic agents may be
useful as a second choice, especially those
in the Ic group. However, if episodes are frequent, recurrent or sustained, or if the patient
prefers a radiofrequency ablation of the slow
pathway may be performed, with a success
rate over 90% with minimum complications
(the risk of causing complete AV block is 0.4%,
and it is usually temporary, although it may require pacemakers on occasions).
Focal Atrial Tachycardia
Regular tachycardia in episodes which usually start

and finish abruptly, although more gradually than AVRNT or orthodromic,
where before each QRS complex there is a P wave originating at some point
of the atria other than the sinus node, with a dierent morphology than the
sinus (depending on the location of the focus) and a usually prolonged PR. On
11
V1 during sinus rhythm
V1 during common AVRNT
DII during sinus rhythm
DII during common AVRNT
Figure 21. ECG of the same patient in V1 and DII during sinus rhythm and common AV nodal
reentrant tachycardia, showing image of pseudoR in V1 and pseudoS in DII
Figure 23. ECG in Wol-Parkinson-White

syndrome
the left pathways further from the sinus node).

Accessory pathways may be bidirectional, only
retrograde (concealed pathways) or only antegrade. Conduction velocity and refraction vary
with each pathway.
The combination of pre-excitation and paroxysmal tachycardia is called Wol-ParkinsonWhite (WPW) syndrome. It may be related to
Ebsteins disease (birth anomaly more frequently related to WPW) and,
maybe, with mitral valve prolapse and hypertrophic cardiomyopathy.
Figure 22. Lewis diagram showing focal atrial tachycardia with irregular conduction to ventricles
because of varying block degrees in AV node
occasion, the AV node does not tolerate such a high rate and some of the
P waves cannot be conducted to the ventricles, even though the tachycardia
remains (Figure 22).
It may respond to -blockers or calcium antagonists (which also stop the
AV node and obtain a slower ventricular response during tachycardia),
catheter ablation of the anomaly is performed if it does not respond, antiarrhythmic agents may further be tried with moderate ecacy.
Multifocal Atrial Tachycardia

There are at least three P waves with dierent morphology (of dierent
focuses) during tachycardia, with a variable ventricle conduction degree.
It is frequent among patients with worsened bronchopathy, especially
under treatment with theophylline or beta-agonists. It frequently evolves
into atrial fibrillation. Treatment consists of improving the patients pulmonary condition (oxygenation) and removing, if possible, theophylline
and beta-agonists. Calcium antagonists or magnesium sulfate may be
useful.
Pre-excitation Syndromes
This occurs when there is a congenital accessory conductive pathway
(in addition to the AV node) for electric communication between atria
and ventricles which, in sinus, causes early depolarization of ventricles
(it pre-excites them), causing a shortened PR, a slurred upstroke to
the QRS complex (the delta wave, showing the fragment of ventricles
depolarized through the accessory conductive pathway before the sinus
impulse goes through the AV node), and a broad QRS (Figure 23).
There may be evident pre-excitation (especially the right pathways
close to the sinus node) or be inapparent in the ECG (especially in
12
There are two types of paroxysmal tachycardia by AV reentry related

to WPW:
Orthodromic. There is anterograde conduction through AV node
and retrograde conduction through the accessory pathway. They
are the most frequent, and QRS during tachycardia is narrow (with
the exception of bundle branch blocks); the second most frequent is
supraventricular paroxysmal tachycardia.
Antidromic. Anterograde conduction is through the accessory pathway, and retrograde conduction through AV node, therefore QRS is
wide (a maximum preexcitation). It is very rare.
Special accessory pathways:
Mahaim-type. Anterograde conduction only, long conduction
time, and decremental properties (similar to having a parallel AV
node, usually in the right atrium). They only result in antidromic
tachycardia.
Coumel-type. Retrograde conduction only (concealed), long
conduction time. They usually cause sustained tachycardia since
childhood with associated tachycardiomyopathy.
The treatment of acute tachycardia episodes because of AV reentrant
is similar to AVNRT.
Pre-excited Atrial Fibrillation

When an AF appears with WPW syndrome (or a flutter or other quick
atrial tachycardia) and the pathway performs fast antegrade conduction,
it is a medical emergency since an excessively fast conduction through
the accessory pathway may result in ventricular fibrillation (VF). If it is not
Card iology
well tolerated hemodynamically, treatment involves electrical cardioversion. If it is well tolerated, electrical cardioversion will be performed or
anti-arrhythmic drugs from the Ic group or procainamide will be applied.
Intravenous administration of digoxin, calcium antagonists (and probably
amiodarone) is contraindicated, since by slowing node conduction and/or
causing vasodilation with increased reflex in sympathetic tone they may
facilitate conduction through the accessory pathway and VF.
A definitive treatment of accessory pathways is percutaneous catheter ablation of the accesory pathway, which is more eective than
anti-arrhythmic drugs and has low risk, and is therefore the treatment
of choice for patients with tachycardia episodes. In subjects with asymptomatic pre-excitation, clinical observation is advisable, except for hazardous-activity professionals (professional drivers, sport players, etc.)
or according to the patients wishes, when ablation is recommended.
Nonparoxysmal Junctional Tachycardia
It is said to be sustained if it lasts for more than 30 seconds or if it causes

circulatory collapse (nearly always in subjects with organic heart diseases, especially prior acute myocardial infarctions).
Nonsustained VT (< 30 seconds) may also be associated with organic
heart diseases, although less frequently than sustained VT. Nonsustained VT is usually asymptomatic, but SVT usually causes hemodynamic changes and symptoms such as myocardial ischemia, syncope.
The following are distinguished according to QRS morphology:
Monomorphic VT. QRS morphology is the same for all beats.
Polymorphic VT. QRS morphology varies from one beat to the other.
Bidirectional VT. Direction of QRS axes alternates.
The most frequent etiology of monomorphic VT is reentry through viable tissue channels located inside or on the edges of a prior acute myocardial infarction scar. Figure 25.
It is a very rare type of tachycardia, caused by an increase in automatism or triggered activity (afterpotentials) in the AV node. QRS are the
same as the patients basal and there is frequently AV dissociation or
VA conduction. It starts and ends gradually and heart rate varies with
maneuvers aecting the autonomic nervous system (increased with
vagolytic drugs, exercise or catecholamines and decreased with vagal
maneuvers or -blockers). Carotid sinus massage may transiently stop
tachycardia, but does not cause it to disappear.
It is characteristic of digitalis toxicity, catecholamines increase, acute lower
myocardial infarction or myocarditis. Treatment is aimed at the root cause;
electrical cardioversion should not be attempted, especially if the cause is
digitalis toxicity, since it is not eective as far as it is not caused by reentrant.
Ventricular Tachycardia
Ventricular tachycardia (VT) involves the presence of three or more
consecutive beats originating in the ventricles at over 100 bpm. A wide
QRS tachycardia is shown in the ECG (over 0.12 seconds), with AV dissociation (there can be some retrograde P waves), usually starting with
ventricular premature beats (Figure 24).
Scar from prior acute

myocardial infarction
Figure 25. Intramyocardial ventricular tachycardia mechanism layout
Figure 24. Monomorphic ventricular tachycardia
13
In the acute phase of the acute myocardial infarction, which does not
yet have a scar, arrhythmias are the result of myocardial irritability
due to ischemia, i.e., ventricular premature beats, ventricular fibrillation or accelerated idioventricular rhythm associated with reperfusion).
Other diseases causing intramyocardial scars (dilated cardiomyopathy, right ventricular dysplasia, Chagas disease, etc.) may likewise
present monomorphic ventricular tachycardia during their clinical
course.
Other monomorphic VT, called idiopathic, appear in patients without
structural heart diseases (they comprise 10% of VT and have better
prognosis), such as right ventricular outflow tract VT (sensitive to adenosine and -blockers, caused in general by a focus with triggered activity) or fascicular ventricular tachycardia (sensitive to verapamil, usually
caused by reentry between the left posterior fascicle and surrounding
myocardium), whose treatment in recurring cases or cases with poor
tolerance is VT focus or circuit ablation.
Faced with a common tachycardia with wide QRS, a dierential diagnosis between monomorphic sustained VT and supraventricular
tachycardia with bundle branch block (aberrancy) is important, since
prognosis is dierent. Verapamil should not be used to distinguish
them, since it could cause shock or even cardiac arrest in a VT (Table
10).
SUPRAVENTRICULAR TACHYCARDIA
If the bundle branch block is equal to the block prior to tachycardia
VENTRICULAR TACHYCARDIA
If QRS > 0.14 s
If there is AV dissociation or variable retrograde conduction
not improve survival. Other anti-arrhythmic drugs are contraindicated

when there is structural heart disease.
In patients with normal or near normal systolic function (LVEF > 40%)
and good clinical tolerance during monomorphic VT, it is possible to
conduct radiofrequency ablation of the reentrant circuit causing the VT
with high success rates, especially in slow VTs.
However, it is necessary to consider implanting an automatic defibrillator or ICD, with capability to apply painless therapies of overstimulation
or internal electric shocks to restore sinus rhythm, both for secondary
prevention (if systolic function is impaired or arrhythmia tolerance is
bad) and for primary prevention in patients with LVEF below 30% after
at least 40 days following a myocardial infarction and under optimized
medical treatment.
Channelopathies
A modern term which comprises a group of arrhythmic syndromes
caused by dysfunction of ion channels located in the membranes of cardiac cells in patients without structural heart diseases.
Long QT syndrome (LQTS: most frequent channelopathy). It is a
congenital or acquired disorder whereby membrane ion currents
lengthen the duration of the action potential shown in the ECG, consisting of a prolonged QT interval, which facilitates the appearance
of afterpotentials (premature beats) that are transmitted to the rest
of the myocardium with functional reentrant phenomena causing
polymorphic ventricular tachycardia with twisting of the points (Torsades de Pointes or helicoidal ventricular tachycardia), where QRS
are polymorphic, i.e., they vary in width and duration, causing an
oscillation pattern on the basal line similar to a helix (Figures 26
and 27). These tachycardia episodes are very fast and cause syncope
if self-contained, although they may evolve into VF and cause sudden death.
Upper QRS axis

All precordial QRS of the same sign (+ or -)
QRS > 0.12 s and does not match with structured or typical RBBB or
LBBB
Time to R wave peak in DII 50 ms + absence of RS in precordial
Table 10. Data for the ventricular or supraventricular origin

of wide QRS tachycardia
Treatment of sustained monomorphic VT with poor clinical tolerance is

electrical cardioversion. When hemodynamics is not compromised, a
choice can be made between electrical cardioversion or drugs (intravenous procainamide or amiodarone).
Recurrence prevention. -blockers are used, which should be taken by
all patients with prior acute myocardial infarction. Amiodarone may be
useful in specific cases to decrease recurrence risk, although it does
Figure 26. Helicoidal tachycardia

The most frequent cause of acquired LQTS is the use of drugs which
interfere with repolarizing ion currents (especially IKr), usually in
predisposed subjects (women with hypertensive ventricular hypertrophy or perhaps certain genetic polymorphisms which predispose
to prolonged QT). Other causes of prolonged QT intervals are those
listed in Table 11.
Figure 27. Torsades de Pointes episode in patient with severe sinus bradycardia, secondary long QT and ventricular premature beat with probable
afterpotentials that start the episode (evolved into VF and required debrillation)
14
Card iology
Myocardial infarction or other acute

coronary syndromes
ISCHEMIA
ELECTROLYTIC
ALTERATIONS
ANTI-ARRHYTHMIC
DRUGS
PSYCHIATRIC DRUGS
Hypocalcemia
Hypokalemia
Hypomagnesemia
Class Ia
Class III
Tricyclic antidepressants
Haloperidol
Phenothiazine
INTRACRANIAL
PROCESSES
Subarachnoid hemorrhage, intracranial

hypertension
OTHER DRUGS
Macrolides, quinolones, antihistamines.
BRADYARRHYTHMIA
CONGENITAL
LONG QT
Third degree AV block.

Jervell-Large-Nielsen syndrome
(autosomal recessive, deafness)
Romano-Ward syndrome (autosomal
dominant, not deafness)
Table 11. Long QT syndrome eology

Congenital LQTS (less frequent) is a genetic anomaly due to mutations
aecting ion channels with two classic phenotypical expressions: Romano-Ward syndrome (autosomal dominant) and Jervell-Lange-Nielsen
syndrome (autosomal recessive linked to congenital deafness).
Up to 12 genetic types of Romano Ward syndrome are reported according to the aected gene (named from 1 through 12), the first three being the most frequent (Table 12).
SUBTYPE
MUTATION
EFFECT
LQTS 1
Activity K
channels
Exercise
-blocker ICD
Stress
Swimming
LQTS 2
Activity K
channels
Waking up -blocker ICD

Noises
K+ supplements?
LQTS 3
Activity
Na+
channels
Tiredness
Fever
T WAVE
TRIGGER
TREATMENT
ICD shock episodes. If syncope or ventricular arrhythmia persists in

spite of -blocker treatment or if it is not eective, an ICD is prescribed
to reduce sudden death risks.
Congenital short QT syndrome. It is a very rare genetic disorder,
caused by the presence of dierent mutations in potassium channels which increase the repolarizing currents and shorten the action
potential in dierent ways, predisposing the subject to supraventricular arrhythmias (atrial fibrillation) or ventricular arrhythmias
(self-limiting polymorphic tachycardia causing syncope or ventricular fibrillation, and sudden death). The corrected QT is lower than
320 ms and the T is very high and spiky.
These are patients without structural heart diseases and, given the
risk of sudden death, the implantation of an ICD is recommended.
The use of quinidine may help in some cases.
Brugada syndrome (BS). It is a genetic disorder which mainly affects the gene SCN5A, in charge of codifying the cardiac sodium
channel (the same one aected by the congenital LQTS type 3 and
some forms of Levs disease), causing a hypofunction of the sodium
channel. Only 30% of patients present recognizable mutations. Nevertheless, up to eight dierent genes (including calcium and potassium channels) have been known to be involved in some cases of
this syndrome.
These patients present a typical ECG with an incomplete right bundle
branch block, a J point above 2 mm and a descending ST segment
(coved-type) with a negative T wave in V1-V3 (this is a diagnosis ECG,
denominated type I). A pharmacologic test can be carried out on patients with uncertain ECGs, consisting of the intravenous infusion of
flecainide, ajmaline or other sodium inhibiting drugs to force the type
I pattern to manifest itself in actually aected patients (Figure 28).
ICD
Group I drugs?
Table 12. Characteristics of main congenital long QT syndrome subtypes

Treatment of Torsades de Pointes is immediate electrical cardioversion.
During the acute phase it is essential to treat the triggering cause (ion
correction, discontinue drugs, treat the ischemia or bradycardia, etc); it is
also possible to use magnesium sulphate or other steps to produce tachycardia (because this reduces QT interval and recurrent episodes) such as
isoproterenol (only in acquired long QT syndrome because in congenital
cases this is contraindicated) or implanting temporary pacemakers.
Drugs and situations which prolong QT should be avoided to prevent
recurrence. Further, -blockers should be used with congenital LQTS
(although they cause bradycardia, they decrease the risk of Torsades de
Pointes), which are very eective in type 1 and also very useful in type 2.
In type 3, the benefit of -blockers is less clear and anti-arrthymic drugs
from group I could be used, such as mexiletine or flecainide, to reduce
Figure 28. Eect of ecainide infusion on the ECG of a patient with

Brugada syndrome. Nondiagnostic basal ECG
15
The arrhythmia associated with Brugada syndrome is a polymorphic

VT which may cause a ventricular fibrillation, usually during sleep,
fever of after using group I anti-arrhythmic agents, or other drugs
blocking sodium currents, such as tricyclic antidepressants. Therefore, it is a common cause of sudden death. It is more lethal in males
and more common in Southeast Asia.
The treatment of symptomatic patients (syncope or sudden death)
consists of avoiding any triggering events and implanting an ICD.
Whether or not an ICD must be implanted in all asymptomatic patients remains a controversial issue, especially when dealing with
relatives of patients with an ECG pattern that can only be unmasked
with drugs, since the risk of long term eects is not high.
Catecholaminergic polymorphic ventricular tachycardia. Presents
itself mainly as the result of mutations in the ryanodine receptor
gene (RyR2). Ryanodine is a protein in charge of transporting calcium from the sarcoplasmic reticulum. It causes polymorphic VT episodes triggered by physical exercise or stress. The bidirectional VT
is also common in this syndrome. -blockers may be useful in these
cases, although they usually require the implantation of an ICD.
Accelerated Idioventricular Rhythm

The accelerated idioventricular rhythm (AIVR) is a ventricular rhythm
resulting from myocardial irritability with ectopic automaticity, the fre-
quency of which usually oscillates between 60 and 120 bpm. It usually

occurs in the context of an AMI and is frequently a sign of reperfusion.
It is usually transitory and only produces major symptoms or hemodynamic alterations under exceptional circumstances, wherefore it generally requires no treatment. In cases with hemodynamic instability it
must be treated with atropine by intravenous infusion (as the cardiac
rhythm increases the sinoatrial node hides the AIVR).
Flutter and Ventricular Fibrillation

These kinds of arrhythmias cause an immediate loss of consciousness
and, if not treated shortly thereafter, death (Figure 29).
It is most frequently caused by a myocardial ischemia, either during
the acute phase (primary VF) or as the result of the degeneration of a
sustained monomorphic VT in a patient who suered a previous acute
myocardial infarction. It is traditionally understood that a primary VF
during the acute phase of the AMI does not aect the long-term prognosis of the patient, since the risk of a relapse is low where no other
episodes of ischemia occur. Nevertheless, some data link this to higher
mortality rates in the short term, not as the result of the arrhythmia
itself (provided the possibility exists to perform immediate defibrillation and CPR), but because this is more usually present in patients with
larger infractions, with a more severe ventricular dysfunction and hemodynamic alteration, and who respond less to reperfusion therapies.
Figure 29. 12-lead ECG of a ventricular brillation. The rhythm strip (lower section) shows the recovery of sinus rhythm after a discharge
of the debrillator implanted on the patient
16
Card iology
When this occurs long after an AMI, it does entail a poor long-term
prognosis. In all other cases recurrence is frequent and a definite ethologic treatment must be scheduled or, where this is not possible, a defibrillator must be implanted.
Sudden Cardiac Death

This is defined as a cardiopulmonary arrest occurred within the first hour
following the onset of symptoms of the disease. It is most frequently
caused by an ischemic cardiomyopathy. Other important diseases that
may cause a sudden death are the dilated and hypertrophic cardiomyopathies (this is deemed to be the most frequent cause of sudden death
among competition athletes, even though data has been collected in Europe suggesting that right ventricular dysplasia can be at least as important as the hypertrophic cardiomyopathy, or more), arrhythmogenic right
ventricular cardiomyopathy, channelopathies, or WPW syndrome. Figure
30 shows the main causes of sudden death.
Therapeutic hypothermia may improve the brain prognosis of some patients resuscitated after a sudden death event.
The implanted cardioverted defibrillator (ICD) is a very useful tool to
prevent arrhythmic sudden death. The current indications are detailed
in Table 13.
CLINICAL STATUS
Secondary Aborted sudden
prevention death
Sustained
monomorphic
ventricular
tachycardia (SMVT)
Primary
Nonsustained
prevention Ventricular
Tachycardia (NVT)
Special
cases
Documented VF or VT because of
irreversible causes
SMVT with hemodynamic
compromise or in patients with
ventricular dysfunction (LVEF
< 40%)
NVT in patients with ischemic
ventricular dysfunction (LVEF
< 40%) with induced SMVT or VF
in the electrophysiology study
Post-AMI left
ventricular
dysfunction
Consider ICD for primary

prevention when EF < 30% at least
40 days after the AMI
Systolic heart
failure
Consider ICD in patients with EF

< 35% and functional class II-III
of the NYHA classification despite
an optimal medical treatment
Consider ICD resynchronization
when LVEF < 35%, LBBB,
asynchrony and NYHA III-IV
despite optimal medical
treatment
Channelopathies
Symptoms (syncope or aborted

sudden death)
Congenital long QT where the
patient suffers a syncope despite
the use of -blockers
Hypertrophic
cardiomyopathy
Several sudden death risk factors
Dilated
cardiomyopathy
Syncope, sustained VT or aborted

sudden death
(see the applicable chapter)
Table 13. Indications for the implantation of an ICD
Heart Failure
Chapter 04
Figure 30. Main causes of sudden death

The treatment for sudden death is immediate cardiopulmonary resuscitation (CPR). When this originates in the heart (most cases), recent data
reveal that thoracic compression maneuvers and access to immediate
defibrillation must be prioritized over other therapeutic measures (including mouth to mouth resuscitation) during basic CPR.
The concept of heart failure (HF) encompasses all conditions in which

the heart is not capable of supplying all of the blood the body needs at
a given time or does so at the expense of causing high ventricular pressures. That is, the cardiac output is insucient to cover all of the bodys
metabolic needs or this is achieved by the generation of diastolic pressures that can produce symptoms by retrograde congestion.
17
4.1. Classification
4.2. Pathophysiology
and Clinical Characteristics

A classification by the American Heart Association/American College of Cardiology and very broadly used nowadays consists of graduating HF states. This provides an idea of how the disease evolves,
dierent from the functional classification of heart failure (New York
Heart Association (NYHA). Class I: no symptoms and no limitation in
ordinary physical activity. Class II: mild symptoms and slight limitation during ordinary activity. Class III: marked limitation in activity
because of symptoms. Class IV: severe limitations and symptoms
even while at rest). This classification is based on the idea that many
patients are at risk of suering symptomatic heart failure which may
be prevented or alleviated with adequate cardiovascular prevention
measures (Table 14).
STAGE A
High risk of heart failure. Unidentified structural or

functional anomaly without signs or symptoms
STAGE B
Structural heart disease clearly developed as the result of

a heart failure, but without signs or symptoms
STAGE C
Symptomatic heart failure associated with an underlying

structural disease
The pathophysiology underlying a heart failure is showed in Figure 31

below.
Ischemic C.,
cardiomyopathies,
etc.
Hypertrophy
apoptosis
fibrosis
Myocardial lesion
Lower O2 inflow
to tissues
(anterograde
symptoms)
Increased O2
requirement
Increased
postload
Advanced structural heart disease and symptoms of heart

STAGE D failure at rest despite receiving the maximum allowed
medical treatment
Perfusion
pressure
Activation
of neurohormonal
systems and inflammation
Renin-angiotensin
aldosterone
Endothelin
ADH
Sympathetic system
Hydrosaline
retention
Natriuretic
peptides
Table 14. Stage classication by the American Heart Association/

American College of Cardiology
The reference to the nature of the predominant functional disorder enables researchers to dierentiate between systolic HF and diastolic HF,
even though the current trend is to replace systolic HF and diastolic
HF with HF with reduced systolic function (reduced LVEF) and HF
with a preserved systolic function (preserved LVEF). This dierence is
essential when it comes to deciding on the appropriate treatment for
each patient (Table 15).
preload
(retrograde
symptoms)
Figure 31. Pathophysiology of heart failure

The symptoms accompanying heart failure are justified in the following
diagram (Figure 32).
HF WITH REDUCED LVEF
HF WITH PRESERVED LVEF
Prevalence
Greater (descending)
Lower (increasing, especially in the elderly)
LVEF
< 50%
> 50%
Left ventricular
dilatation
Yes
No
Left ventricular
hypertrophy
Scarce
Frequent
Treatment
Diuretics (were not proven to improve prognosis)

ACE inhibitors, ARA II or hydralazine + nitrates, LCZ696 (sacubitril)
-blockers
Spironolactone, eplerenone
Consider digoxin, ICD and CRT
Avoid verapamil, diltiazem and anti-arrhythmic agents (except
amiodarone)
Ivabradine (HR > 70)
-blockers
Verapamil or diltiazem
Careful preload and afterload management
(diuretics and vasodilators)
Usual etiologies
Ischemic cardiopathy
Dilated cardiomyopathy
Valvular heart disease
Myocarditis
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
Hypertension and diabetes
Pericardial disease
Table 15. Dierences between heart failure with depressed systolic function and heart failure with preserved systolic function
18
Card iology
LEFT CONGESTION (retrograde failure)
RIGHT CONGESTION (superior vena cava):
Dyspnea;
Orthopnea;
Paroxysmal nocturnal dyspnea;
Acute pulmonary edema
Jugular distension
LEFT ANTEROGRADE INSUFFICIENCY

HYPOPERFUSION:
Peripheral (limb coldness);
Renal ( aldosterone, fluids);
Cerebral (confusion, memory alterations)
RIGHT CONGESTION (inferior vena cava)
RIGHT ANTEROGRADE INSUFFICIENCY
Edema in declining zones.

Painful hepatomegaly,
cirrhosis, varicose veins
Left ventricular loading defect
Figure 32. Heart failure symptoms and their pathophysiologic origin

The clinical alterations that occurred as the result of a heart failure are
summarized in Figure 33.
4.3. Complementary Tests

ECG can reveal nonspecific alterations or alterations suggesting the etiology of the clinical condition (Q waves demonstrating previous AMI,
cavity enlargement, supraventricular arrhythmia such as atrial fibrillation, etc.).
Chest x-ray (Rx) can show cardiomegaly and signs of pulmonary venous hypertension, such as vascular redistribution, signs of a peribronchial, perivascular and alveolar edema, a pleural or intercisural
effusion. Acute pulmonary edemas show a butterfly-shaped diffuse

perihilar bilateral pattern of alveolar infiltrate (Figure 34).
An echocardiography must be performed on all patients with clinical symptoms suggesting a heart failure (first episode), since this frequently enables diagnosing the etiology and provides useful data for
prognosis purposes. The echocardiography must be conducted to find
global or segmental structural heart anomalies causing the failure, and
to determine global systolic function by means of the estimation of the
LVEF. The presence of anomalies in the regional wall contraction is very
common among patients with an ischemic etiology. Other elements
frequently observed are the dilation and increased sphericity of the
ventricles (especially the left ventricle), mitral and tricuspid regurgitation secondary to the dilation of the rings and various data depending
on the etiology (serious hypertrophy in patients with high blood pressure, aneurysms in some patients with previous AMI). Diastolic func-
19
Figure 34. Radiology of acute pulmonary edema (APE): buttery-shaped

alveolar inltrates
4.4. Treatment
of Heart Failure
Figure 33. Clinical symptoms of congestive heart failure

tion may be analyzed through dierent echocardiographic-Doppler
techniques, the most common one of which is analysis of mitral inflow.
A magnetic resonance of the heart enables detection of morphologic
alterations more precisely than an echocardiogram, and enables estimation of masses, volumes and LVEF with the outmost exactitude. An
isotopic ventriculography can provide data on systolic dysfunction and
applicable volumes.
A dilutional hyponatremia is usually a late sign of a heart failure, and is
therefore usually associated with a poor prognosis.
Brain natriuretic peptide (BNP) can be used for diagnosis purposes
and usually forecasts HF. Natriuretic peptides are released as the result of sodium overcharge or stretching of the muscular fibers, and
produce sodium and water excretions (inhibits reabsorption in the
proximal tubule), and arteriolar and venous vasodilation (countering
the eects of angiotensin II, vasopressin and sympathetic stimulation), therefore lowering peripheral vascular resistances.
A clinical test can directly determine this factor or the NT-pro-BNP, with
a longer average life which is therefore more stable. Certain conditions,
such as those present in the female organism, renal failure, an COPD, a
pulmonary embolism, or old age can increase the BNP level, and obesity
can lower it.
20
The treatment of heart failure includes, initially, the correction of the

underlying cause, if possible (AMI, valvular heart disease, constrictive
pericarditis, etc.) and/or of the triggering events (hypertensive emergency, arrhythmias, infections, anemia, etc.). The presence of triggering events is very frequent in patients with a previously diagnosed and
stable HF, and is a key factor, since the identification and treatment of
a triggering event can improve the prognosis of a patient who suered
a HF.
General Therapeutic Measures

Instruct the patient to participate actively in their treatment and
comment on the importance of completing all therapeutic regimens. The patient can be taught to modify the treatment, especially the dosage of diuretics depending on their volume state.
Restrict the intake of salt. Restrict the intake of fluids to less than
1.5-2 L/day in very ill patients or hyponatremia.
Limit consumption of alcohol to 10-20 g/day (entirely if the patient
has suered an alcoholic cardiomyopathy).
Obese patients (BMI > 30) should be advised to lose weight, but this
should not be recommended to all patients, since cardiac anorexia
and cachexia are frequent problems.
Patients should be advised to quit smoking. Vaccination against influenza and pneumococcus should be considered in the absence of
contraindications.
Patients should be advised to perform quite moderate physical activities adapted to functional situation.
Sleep apnea/hypopnea must be investigated and treated when
present.
Card iology
Associated depression problems should be investigated and treated.

The use of nonsteroidal anti-inflammatories and COX-2 inhibitors,
corticosteroids, type I anti-arrhythmic agents, calcium antagonists
such as verapamil, diltiazem, or nifedipine, tricyclic antidepressants,
and lithium salts should be avoided except under exceptional circumstances.
Treatment of a Con irmed HF

It is based on several actions (Figure 35).
Symptomatic treatment
Symptomatic
dysfunction
ACE inhibitor
ARA II
Beta blockers
Diuretics
H. failure
Digoxin
Nitrates
Hydralazine
Spironolactone
Severe H. failure
Heart transplant
Prevention of deterioration
ACE inhibitor
Beta blockers
Ivabradine?
Advanced stages
Spironolactone
Cardiac resynchronization
Ventricular assistance
Figure 35. Evolution of the treatment of heart failure

There is increasing evidence of the importance of approaching patients
in a global manner, with multidisciplinary teams (cardiologists, general
physicians, Primary Care providers, nurses, etc.), sanitary education,
self-care and early decompensation detection programs, among others.
The assessment and treatment of any existing comorbidities likely to
precipitate or worsen HF or alter the patients quality of life, such as
anemia, iron deficiency, renal failure, sleep apnea, erectile dysfunction,
depression, etc., is of outmost importance.
Prevention of the Deterioration

of the Heart Function
A HF activates dierent neurohumoral systems which attempt to reestablish the adequate perfusion of tissues but unchain deleterious effects in the long term. The most important progress in the treatment of
HF patients over the last years focused on the use of drugs antagonizing these systems (renin-angiotensin-aldosterone system, sympathetic
nervous system)
ACE inhibitors. Given that these are mixed vasodilators (arterial and venous), they lower preload and afterload, and favor increased output of the heart. Their use is associated with an improvement of the functional class. Several studies have revealed
an increase in survival rates (enalapril, captopril, ramipril), both
in patients with HF of ischemic etiology and in patients with car-
diomyopathies with other etiologies in the dilation stage. They

also lower the possibilities of asymptomatic patients with left
ventricular dysfunction to develop HF. These drugs can reduce
the seriousness of events (death, AMI or CVA, HF and diabetesrelated complications) in subjects with a high risk of suering
heart diseases, which is more evident in diabetic patients. As an
alternative, patients developing cough and angioedemas from
ACE inhibitors can be prescribed antagonists of the angiotensin
II receptor. A new treatment, LCZ696, has been recently proven
to work better than enalapril in patients with HF and LVEF < 40%
to prevent the morbimortality associated with HF. LCZ696 is a
combination of valsartan, ARA II, and sacubitril, an inhibitor of
neprilysin, also known as neutral endopeptidase, a zinc-dependent metalloproteinase in charge of the deactivation and cleaving of several endogenous peptides, including amyloid-beta protein.
Beta blockers. Together with ACE inhibitors and spironolactone,
these are the pillars of the current treatment of patients with HF.
Several studies have shown them to improve LVEF and functional
class, lower hospitalization rates, and increase the survival rates
of patients with HF (preventing both sudden death and the death
resulting from the progression of the HF). The drugs on which
conclusive data have been obtained are carvedilol (the most
studied and used), metoprolol, bisoprolol and nebivolol.
The treatment must be commenced with low doses which are to
be increased slowly since, given that these are negative inotropic drugs, they can initially worsen the heart failure. Their use is
admitted for all of the functional classes reported by the NYHA
(I to IV), provided the patient is euvolemic, which can usually be
obtained by means of a concomitant treatment with diuretics.
Mineralocorticoid receptor antagonists. Spironolactone and
eplerenone. Spironolactone is a potassium-sparing diuretic acting in the distal tubule, an aldosterone antagonizing drug. Their
beneficial eect on HF patients is not the result of their diuretic
eect, but rather its antagonism of the deleterious eects of high
aldosterone levels: vascular fibrosis, sympathetic activation, reduction in arterial distensibility, increased sodium in the body. Its
use at low doses has been shown to increase survival rates, decreasing both total mortality and sudden death rates in patients in
advance functional class of the NYHA (III and IV). They should not
be prescribed where creatinine values are higher than 2.5 mg/dL
or potassium levels are above 5 mEq/L, due to the risk of hyperkalemia.
Eplerenone (a more selective inhibitor than aldosterone) has
been shown to increase survival rates in patients who have suffered an AMI and present a LVEF over 40% and clinical signs of
HF or diabetes the first days following a myocardial infarction. In
patients with a good functional class (class II of the NYHA) eplerenone appears to be useful to lower the combined cardiovascular
mortality endpoint or hospital admission as a result of HF exacerbation. Its main advantage as compared to spironolactone is
that it causes a lower gynecomastia rate, even though it is more
expensive.
Reduction of Cardiac Work

Reduction of afterload. The use of hydralazine and nitrates is an effective alternative to ACE inhibitors in intolerant patients, since said
21
combination has also been proven to increase survival rates, especially among black patients, who have obtained better prognosis, in
addition to the treatment with ACE inhibitors and beta blockers in
patients who remain symptomatic.
Reduction of preload. Diuretics reduce the preload and the pulmonary and systemic congestion symptoms, whereby they are extremely useful in cases of decompensation. Figure 36 shows the
action mechanism of the dierent kinds of diuretics.
However, they have not been proven to increase survival rates (with
the exception of spironolactone, but not because of its diuretic effects, as mentioned above). The main kinds of diuretics are shown in
Table 16.
Torasemide has been shown to have antifibrotic eects in experimental
models.
It must be taken into account that the excessive use of these drugs
can exacerbate the sensation of weakness as the result of the lowered cardiac output (by excessively reducing the preload necessary
to maintain the Frank-Starling pressure-volume loop at its optimal

values) and cause water-electrolyte imbalances (hypokalemia and
hyponatremia).
Nitrates (predominantly venous vasodilators) also improve the symptoms of pulmonary congestion, as they reduce preload but have no
proven eect on survival rates.
Reduction of heart rate. An elevated heart rate is a risk factor, in
both healthy individuals and coronary or HF patients with a depressed systolic function. Reducing the heart rate also reduces
the cardiac work rate, which explains some of the beneficial effects beta blockers have on patients with heart failure. A recent
study has assessed the eects of the reduction of the sinoatrial
heart rate in addition to the one achieved with beta blockers
by means of the use of ivabradine, a selective blocker of the
If current in the sinoatrial node, in patients with a sinus rate
over 70 bpm, with an ejection fraction below 35% in functional
class II-IV. The use of this drug has been proven to lower hospitalization and mortality rates because of heart failure, as compared to placebo.
Figure 36. Site of action and mechanism of diuretics
22
Card iology
DIURETIC
ACTION MECHANISM
INDICATIONS
CONTRAINDICATION
COLLATERAL EFFECTS
Thiazides (chlorothiazide,
xipamide, indapamide)
Inhibition of Na/Cl
channels in distal
convolute tubule
Mild or moderate heart

failure
Hypertension
When GF < 25 mL/min.
Hypo K
Hyperuricemia
Increased glucose and
lipids
Loop diuretics (frusemide,

etacrynic acid, bumetanide,
indacrinone)
Inhibition of the
Na+-K+-2Cl- symporter
(cotransporter) in the
thick ascending limb
of the loop of Henle
Anuria
Pregnancy
Hypersensitivity
K+ sparing agents
1. Aldosterone antagonists
(spironolactone)
2. Amiloride, triamterene
Blocks the action of

aldosterone in distal
convolute tubule
Hyper K
Associated with other
diuretics with edemas or HTN Renal failure
Treatment with K
Hyperaldosteronism not
supplements
correctible with surgery
Edemas with
hyperaldosteronism
(nephrotic syndrome, CHF,
cirrhosis)
Impotence,
gynecomastia
Hyper K
Blocks the absorption

of Na+ in distal
convolute tubule
Same as above
Liddles syndrome
Hyper K
Refractory edema
Serious acute edemas
GF < 25 mL/min.
Serious HTN
Hypo K
Hypo Na
Hyperuricemia
Hypo Mg
Cytotoxicity
Table 16. Main characteristics of main diuretic types
Increase in Myocardial Contractility: Inotropes
cially, beta blockers (improvement in survival), although their use

exceeds their anti-arrhythmic eect.
Digoxin. Has been traditionally used to treat combinations of atrial fibrillation and heart failure, even though no clinical trials have
proven its eects on survival rates. It has also not been proven to
increase survival rates in sinus rhythm, but does reduce hospitalization rates in cases of heart failure.
It must not be used in patients with HF and preserved ejection fraction (unless the patient needs their heart rate controlled by AF).
The beneficial eects of digoxin are believed to result, partially,
from the increase in vagal tone resulting from its use, since the
levels useful in treatment (between 0.6 and 1.2 g/mL) are more
related to this eect than to the increase in contractility. Its therapeutic margin is very low, so it is common for drug toxicity to occur
(Figure 37).
Sympathetic agonist drugs. Dopamine and dobutamine can be used
in decompensations in which patients do not respond to conventional therapy. Ibopamine is an oral dobutamine analogous drug
which alleviates symptoms but also worsens the prognosis of HF
patients, whereby it is not normally used.
Phosphodiesterase III inhibitors. They have also not been shown
to cause an assessable improvement in patients with heart failure.
Levosimendan. Calcium sensitizer which may be used instead of
sympathetic agonist drugs in cases of decompensations requiring
inotropic support.
Implantable Devices for Cardiac Rhythm Treatment

In general, anti-arrhythmic drugs are contraindicated in heart failure patients caused by a systolic dysfunction of the left ventricle.
When such patients suer arrhythmias, amiodarone or dofetilide
may be used (both have a neutral impact on mortality) and, espe-
Digoxin toxicity
No digoxinemia
Digoxinemia
K+, Mg2+, O2,

pH, thyroids,
Ca2+
Renal failure
Drugs
Digestives
Electrical
Nausea, vomiting, diarrhea
More frequent: ventricular extrasystoles

Atrioventricular block
More specific: supraventricular tachycardia
with variable AV block
Accelerated nodal rhythm
Bidirectional tachycardia
Figure 37. Digitalis toxicity

The implantable cardioverter-defibrillator (ICD) has become a
device widely used in patients at high risk of sudden death, especially for ischemic heart disease. ICD should be indicated patients
with HF who have been resuscitated from cardiac arrest (secondary prevention), patients that present symptoms (usually syncope
or pre-syncope) related to ventricular arrhythmias, and patients
with systolic dysfunction of an ischemic nature for whom ventricular tachycardia has been induced in an electrophysiologic study.
ICD use for primary prevention is accepted in patients that present severe ventricular dysfunction (LVEF below 35%) and NYHA
functional class II and III, despite optimal medical treatment. ICD
23
may also be indicated for NYHA functional class I patients if LVEF

is below 30%, but only if etiology of the ventricular failure is ischemic.
Cardiac resynchronization therapy (CRT) aims to improve the mechanical asynchrony in a high percentage of patients with advanced
systolic dysfunction, presenting a wide QRS (usually because of a left
bundle branch block, secondary to fibrosis, of the impaired left ventricle conduction system) responsible for a substantial ventricular
contraction ineciency.
This therapy is based on the use of a pacemaker that stimulates both
ventricles simultaneously, in order to maintain a synergy in the contraction of the left ventricle walls, thus obtaining more eective pumping. This is accomplished by placing one stimulating lead in the apex of
the RV and lead in the lateral or posterolateral epicardium of the LV,
generally through the coronary venous system. It is indicated for patients with severe ventricular dysfunction (LVEF below 35%), especially
in sinus rhythm, for symptomatic patients (NYHA functional classes II,
III or IV outpatient cases), despite adequate medical treatment, with
confirmed asynchrony (QRS wider than 150 ms [especially in case of a
left bundle branch block, LBBB]). In this context, CRT has demonstrated
that it improves survival, functional class, exercise tolerance and the
QOL of patients. In patients with dierent characteristics (atrial fibrillation, QRS between 120-150 ms, right bundle branch block) benefits are
less clearly defined and indication for CRT should be carefully assessed
in each case.
Indications for ICD and CRT frequently overlap; therefore, the current trend
is to implant an CRT-ICD: ICD that enables application of CRT (Figure 38).
When diastolic function is impaired, the atrial contribution to ventricular filling becomes more important, therefore it is useful to treat
atrial fibrillation by maintaining sinus rhythm as long as possible.
In case it is necessary to implant a pacemaker, it should be a sequential
(bicameral) pacemaker in order to maintain atrioventricular synchrony.
Relaxation of the ventricular myocardium (lusitropic eect) may be
enhanced by decreasing the intracellular calcium concentration. This
may be indicated for patients with HF and preserved LVEF and can be
achieved with two types of drugs: beta blockers and calcium channel
blockers, verapamil and diltiazem (except for patients with systolic dysfunction). These drugs have an anti-angina eect, a relevant fact considering that an impaired diastolic function often appears as a consequence of myocardial ischemia.
Angiotensin receptor blockers, ARBs (candesartan) could contribute to
reduce hospitalizations. ACE inhibitors may also be used. Treatment
with diuretics and vasodilators should not be too rigorous for patients
with diastolic dysfunction as it is necessary to maintain an adequate
preload to prevent a decrease in cardiac output.
Anticoagulation for Heart Failure

Oral anticoagulant therapy may be indicated in patients with HF, especially those with atrial fibrillation, atrial or ventricular thrombus, or patients who had a stroke in the past.
Future Strategies for Heart Failure Treatment

Gene therapy and the introduction of myoblasts or stem cells in the
myocardium, with the aim of regenerating damaged muscle, are interesting research lines currently ongoing.
Acute Cardiogenic Pulmonary Edema Treatment

Cardiogenic pulmonary edema is a medical emergency that requires
careful monitoring in order to maintain blood pressure, heart rate
and urine output and, occasionally, the pressure of the pulmonary
vessels by means of a Swan-Ganz catheter. Treatment is to be quickly
commenced.
Figure 38. Cardiac resynchronization therapy device
Diastolic Dysfunction Treatment

CI with preserved ejection fraction and with no valvulopathy or heart
disease to justify HF is attributed to an impaired diastolic function and,
therefore, therapeutic actions should be aimed at improving ventricular
filling (diastolic function). High heart rate should be avoided and should
be maintained between 60-90 bpm.
24
HF with depressed LVEF is the most commonly found underlying disease. In this case, treatment includes:
High-flow oxygen. Noninvasive or invasive mechanical ventilation
could be performed if necessary.
The patient should be seated, if possible, legs dangling.
Morphine sulfate improves the symptoms because of vasodilatory
and central sedative eects.
Potent diuretics should be used, such as furosemide, which also has
a vasodilatory eect.
Vasodilators should be used when blood pressure is not low (SBP
> 90-100 mmHg). Nitroglycerin is often administered intravenously,
but in cases such as pulmonary edema secondary to acute mitral or
aortic regurgitation, more powerful vasodilators are preferred, due
to their eectiveness on the arteries, such as intravenous nitroprusside, a drug that should always be used in an intensive care unit with
invasive monitoring of blood pressure.
Arterial hypotension may be treated with an intravenous positive
inotropic agent, such as dopamine, dobutamine or levosimendan.
Card iology
An intra-aortic balloon pump may be useful in some cases, especially when blood pressure is low.
position which, by means of a percutaneous connection, which enables

battery recharge (artificial heart).
Note that the use of sympathetic agonist drugs tends to worsen the
situation if the underlying heart disease is a condition with pure diastolic dysfunction, such as mitral stenosis or hypertrophic obstructive cardiomyopathy, and the triggering agent of the acute pulmonary edema is atrial fibrillation with rapid ventricular response (a
relatively frequent event). Nevertheless, beta blockers or calcium
channel blockers may be useful, as they prolong diastolic time by
slowing conduction through the AV node and have lusotropic eects.
However, an urgent electrical cardioversion is usually indicated in
this case.
With these devices, patients may be discharged in order to wait for their
heart transplant at home, or they may serve as therapy for patients not
eligible for transplantation. Nonetheless, their long-term use is associated with a significant risk of complications like bleeding, embolism and
infection (Figure 40).
Severe Myocardial Failure Treatment
Another alternative mechanical support for patients with very severe

cardiac insuciency is extracorporeal membrane oxygenation (ECMO),
which involves the removal of nonoxygenated blood from the superior
vena cava, its oxygenation inside the device and its reinfusion to the
inferior vena cava (veno-venous ECMO, respiratory support only) or to
the arterial territory (veno-arterial ECMO, respiratory and circulatory
support).
Intra-aortic Balloon Pump

It is an eective percutaneous assisted circulation technique that can be
implanted in conscious patients. A catheter with a balloon is introduced
in the femoral artery and pushed towards the thoracic aorta, placing
the balloon immediately distal to the left subclavian artery.
The balloon is inflated during diastole and deflated during systole.
ECG signal and invasive pressure should be monitored in order to
synchronize the inflation and deflation. This process increases cardiac
output by 0.5 or 0.7 L/minute, thus increasing myocardial perfusion
during diastole with concomitant afterload reduction during systole.
Correct use of this procedure results in decreased systolic blood pressure and increased diastolic blood pressure (thus raising mean blood
pressure).
Contraindications: significant aortic insuciency (AI), aortic dissection,
and access problems because of peripheral vascular disease.
An IABP is mainly indicated for patients in cardiogenic shock, patients that present low cardiac output after cardiac surgery, those
with unstable angina resistant to medical treatment in preparation
for emergency coronary angiography, those
facing mechanical complications after acute
myocardial infarction and in very ill patients
as a bridge to heart transplantation (Figure
39).
Figure 39. Intra-aortic balloon pump
Ventricular Assistances and Artificial Heart

Ventricular assist devices provide myocardial
support. For that purpose, blood is drawn from
an atrium or vein and sent through the corresponding artery (this may be a pulmonary or
aortic artery, and it may consist of right ventricular assistance, left ventricular assistance,
or both at once). They are implanted in operating rooms and are indicated upon cardiogenic
shock after surgery, as a bridge to heart transplantation and in some cases of severe acute
myocarditis until actual recovery.
Dierent models with dierent types of operation exist. Some devices are fully implantable
inside the body, and placed in a preperitoneal
Figure 40. Ventricular assistance
25
Heart Transplantation
disease or sudden death. Other causes include tumors (skin cancer and
lymphoma being the most frequent) or acute rejection.
This is a very eective therapy for the treatment of end-stage heart failure. Results are good, not only regarding survival but also regarding lifequality (80% in functional class I). Current survival rates after one and
five years are over 80% and 70%.
Indications and contraindications are summarized in Tables 17 and
18.
According to the standard surgical technique, the donor heart is removed by severing the veins (cava and pulmonary) and large vessels
(pulmonary artery and aorta), therefore, the atria remains intact. The
heart of the recipient is removed by cutting o near the atrioventricular
groove, thus maintaining a portion of both atria. The implant is performed by the removal of donors posterior atrial wall and its subsequent suture to recipients atria. The pulmonary artery and the aorta
are subsequently sutured. Other less-used techniques exist, such as the
bicaval technique. Notwithstanding myocardial protection with cardioplegic solution (rich in potassium) and cold, ischemic times should be
below 5 h (see Figure 43).
The most common cause of death after heart transplantation is infection (17%). However, acute graft failure is the most common cause of
death during the first month, and, in the long term it is graft vascular
Hyperacute rejection is unusual and consists of severe endothelial injury due to preformed antibodies to HLA or AB0 systems.
Lymphocytic infiltration may occur upon acute rejection, which generally appears a week from transplantation and before the first year.
Although it may manifest as heart failure, outstanding clinical signs
are rare, and only minor symptoms may manifest, such as fatigue,
low-grade fever, arrhythmias, or the case may be even asymptomatic.
In most medical centers, acute rejection is studied by transjugular biopsy further spaced over time.
During transplantation follow-up, an endomyocardial biopsy is performed
periodically in order to supervise rejection. There is no other noninvasive
technique able to match the performance of endomyocardial biopsy.
Chronic rejection occurs further in time and involves damage to the
coronary circulation. Repeated rejection episodes seem to favor ocRELATIVE
ABSOLUTE
(Suitable patients in advanced functional class)
Refractory cardiogenic shock
Documented dependence on intravenous inotropic
support to maintain adequate tissue perfusion
Peak oxygen consumption below 10 mL/kg/min
once the anaerobic threshold has been reached
Uncontrollable ventricular arrhythmias despite all
possible therapeutic measures
Constraining ischemic symptoms, refractory
to therapeutic treatments (which include
revascularization)
Patients with congenital heart disease in advanced
functional class ineligible for surgical correction
The most dangerous complication in the early postoperative stage is

right heart failure due to pulmonary hypertension. Selective pulmonary
vasodilators (nitric oxide, sildenafil, prostacyclin) may be used, but generally, results are not good.
INSUFFICIENT
Peak oxygen consumption from 11

Severely depressed LVEF
to 14 mL/kg/min (or below 55% of predicted) Functional class III to IV
in advanced functional class
Peak oxygen consumption above
Persistent ischemia incurable by any other
15 mL/kg/min (or above 55%
intervention
of predicted)
Recurrent instability in hydrosaline balance,
despite adequate therapeutic treatment
Table 17. Indications for heart transplantation
AGE
> 65 years of age (relative)
AB0 INCOMPATIBILITY
INFECTIONS
MALIGNANT NEOPLASMS
LUNG DISEASES
SEVERE KIDNEY/LIVER FAILURE

MULTISYSTEM DISORDER WITH
MULTIORGAN INVOLVEMENT
OTHERS
Absolute: sepsis, active AIDS, HCV and HVB

Relative: active infection
Up to five years after complete remission
Severe COPD or restriction
Recent pulmonary infarction or thromboembolism
Severe pulmonary hypertension (and mean transpulmonary gradient > 15 mmHg) irreversible using
drugs
Creatinine clearance < 50 mL/kg/min (relative, a combined heart and kidney transplantation may be
considered)
Diabetes with severe lesions
Drug addiction, active alcoholism, lack of cooperation, uncontrolled severe mental illness, severe
peripheral vascular disease or brain disease, active peptic ulcer, other diseases with poor prognosis
Table 18. Contraindications for heart transplantation
26
Card iology
currence, as well as CMV infection and hyperlipidemia (therefore an

aggressive treatment of hyperlipidemia is indicated in transplanted
patients). Both the main coronary arteries and distal branches are affected. There is often a pattern that draws attention to the shortage
or severe reduction in caliber of the secondary and tertiary branches,
since eects are more general (less focal) than those that aect nontransplanted patients. A coronary angiography may underestimate the
severity of intimal thickening, thus the best technique for such diagnosis is intravascular ultrasound (IVUS), while the multi-slice scanner
angiography appears as a promising alternative. Angina appears exceptionally, although there is evidence of reinnervation, at least partially, in
many transplant patients, and may frequently occur as sudden death or
silent infarction.
Immunosuppressant drugs are used as of transplantation (initially, in high
doses as part of the induction therapy): with polyclonal antilymphocyte
antibodies, rabbit antilymphocyte or antithymocyte globulin, monoclonal
antibodies to murine CD3 surface antigen of lymphocytes (muromonabOKT3), or humanized monoclonal antibodies to the interleukin-2 receptor (CD25) in T lymphocytes (daclizumab and basiliximab, are currently
the most widely used drugs because they are not associated with an increase in CMV infections and cause fewer side eects).
The chronic immunosuppressant therapy considered to be standard until
recently included cyclosporine A, azathioprine and glucocorticoids (triple
drug combination). Nonetheless, the combination of tacrolimus (a calcineurin antagonist such as cyclosporine) with mycophenolate mofetil associated with corticosteroids is currently used. Upon progress, addition or
substitution of other immunosuppressants is usual, (proliferation signal
inhibitors) such as rapamycin (sirolimus) or everolimus, which seem to
be useful, especially in those patients with graft vascular disease. Chronic
immunosuppression leads to an increase in infections, tumors and other
complications.
GROUP
Hypertrophic cardiomyopathy
Dilated cardiomyopathy
Cardiomyopathies
Chapter 05
Cardiomyopathy describes the evidence of structural and functional

myocardial abnormalities in the absence of any of these four diseases:
coronary artery disease, valvulopathy, congenital heart disease and hypertensive heart disease, suciently severe as to justify the anomalies
detected (e.g., mild hypertension can justify some degree of left ventricular hypertrophy, but not a severe septal hypertrophy associated
with family history of the disease and high risk of premature sudden
death, which may be justified by a hypertrophic cardiomyopathy).
Within each type, the cardiomyopathies of familial/genetic nature
(whether because of identified mutations or not) are distinguished from
those of nonfamilial/nongenetic nature; the latter can be acquired or
idiopathic, secondary to dierent disorders.
Genes involved in the various kinds of cardiomyopathies overlap to
some degree, i.e. mutations in the same gene, even same mutation,
may lead to dierent types of cardiomyopathies.
Nonetheless, by way of summary, mutations in genes that encode sarcomeric proteins usually produce hypertrophic cardiomyopathy; those
that encode cytoskeletal proteins produce dilated cardiomyopathy;
those that encode intercalated disc-associated proteins produce arrhythmogenic right ventricular cardiomyopathy; and those that cause
storage diseases produce restrictive cardiomyopathy. Table 19 summarizes the main types of cardiomyopathy and its etiology.
TYPE
CAUSES
Familial/genetic
Mutations affecting sarcomeric protein genes

Friedreichs ataxia
Noonan syndrome
Glycogen storage disease (Pompe disease)
Lysosomal disease (Anderson-Fabry disease)
Fatty-acid metabolism disorder
Nonfamilial/nongenetic
Obesity
Infant of diabetic mother
Overtraining syndrome
Familial/genetic
Mutations affecting cytoskeletal, sarcomeric or Z band

protein genes, nuclear membrane genes, intercalated disc
genes and some mitochondrial cytopathies
Infectious myocarditis: Coxsackievirus B, AIDS, diphtheria,

Chagas disease
Alcoholic cardiomyopathy
Arrhythmias (tachycardiomyopathy)
Kawasaki disease
Churg-Strauss syndrome
Pregnancy (peripartum cardiomyopathy)
Chemicals and drugs (anthracyclines, cyclophosphamide)
Endocrinopathies (Myxedema)
Nutritional deficiencies (carnitine, thiamine: beriberi;
selenium: Keshan disease; hypophosphatemia and
hypocalcemia)
Table 19. Etiologic classication of cardiomyopathies (continued)
27
GROUP
TYPE
Restrictive cardiomyopathy
CAUSES
Familial/genetic
Familial amyloidosis (transthyretin, apolipoprotein)

Mutations affecting sarcomeric protein genes
Desminopathies
Pseudoxanthoma Elasticum
Hemochromatosis
Lysosomal disease (Anderson-Fabry disease)
Glycogen storage disease (Pompe disease)
Amyloid Light-chain (AL) amyloidosis (prealbumin)

Scleroderma
Carcinoid syndrome
Postradiation damage
Endomyocardial fibrosis:
- Idiopathic
- Hypereosinophilic syndrome (Loeffler)
- Drugs: serotonin, ergotamine, methysergide, mercurial
agents, busulfan
Anthracyclines (rare)
Metastasis
Cardiac lymphoma
Arrhythmogenic right
ventricularcardiomyopathy
Familial/genetic
Mutations affecting desmosomal protein genes
Not reported
Nonclassifiable cardiomyopathy
Familial/genetic
Spongiform cardiomyopathy (Noncompaction

cardiomyopathy)
Tako-tsubo cardiomyopathy (Apical transient dyskinesia,

apical ballooning) or stress cardiomyopathy
Familial/genetic
Congenital long QT syndrome, Congenital short QT

syndrome, Brugada syndrome, Familial catecholaminergic
ventricular tachycardia
Acquired long QT syndrome
Channelopathies
Table 19. Etiologic classication of cardiomyopathies (continued)
The proposed classification includes genetic disorders that predispose

to the development of cardiac arrhythmias (channelopathies) as a specific cardiomyopathy group, although they do not display significant
mechanical dysfunction in the vast majority of cases, as the mechanical and/or electrical alteration is already considered in the definition
of cardiomyopathy. Furthermore, in this classification, a type of cardiomyopathy is referred to as primary if it almost exclusively aects the
myocardium and as secondary if myocardial dysfunction is just another implication of a clinical condition with multisystem involvement.
The dierent characteristics of three classic cardiomyopathies (hypertrophic, dilated and restrictive), are summarized in Table 20.
HYPERTROPHIC
DILATED
5.1. Dilated Cardiomyopathy

The prevalence of this disease is about 1/2,500 individuals. It is defined
by evidence of left ventricular dilation and dysfunction without abnormal
conditions (such as hypertension or valvular disease) or heart disease, sufficient to cause the observed overall systolic deterioration. Right ventricle
deterioration is not necessary for diagnosis, although this is common in
late stages and is associated with worse prognosis. In a reduced number
of cases (10%) systolic dysfunction does not involve dilation.
When etiology is not detected by means of meticulous investigation
(see Table 20) the case involves an idiopathic
dilated
cardiomyopathy, which occurs in alRESTRICTIVE
most half the cases.
LV volume
=//
LV thickness
= or (slight)
= or (slight)
Dysfunction
Diastolic
Systolic
Diastolic
Ejection fraction
=/
=/ (slight)
Clinical observations
Asymptomatic
SAD symptoms:
- Syncope and
sudden death
- Angina
- Dyspnea
Heart failure
symptoms
Mural thrombus
strokes
Arrhythmias
Angina episodes
are unusual
Effort dyspnea
Right heart failure
Strokes
Table 20. Dierences among hypertrophic, dilated and restrictive cardiomyopathy
28
Apparently, the phenotype of dilated cardiomyopathy may be secondary to genetic disorders (25%) or may as well be the final expression of myocardial damage produced by a large
amount of circumstances that converge in left
ventricular dysfunction and dilation; excessive alcohol consumption is the most frequent
cause of secondary dilated cardiomyopathy.
A considerable proportion of these patients respond to a history of viral myocarditis that may
Card iology
be unnoticed or may have caused mild symptoms: gastroenteritis or a

slight cold. They may be reversed by correcting triggering factors such as alcoholism, tachycardiomyopathy, nutritional deficiency or hypothyroidism.
Mechanical dyssynchrony (because of left bundle branch block), whether spontaneous or produced by chronic stimulation from the right
ventricular apex with a pacemaker, may produce a progressive systolic
dysfunction that imitates the dilated cardiomyopathy, and may be prevented or reversed with cardiac resynchronization therapy.
This primarily aects young individuals, especially males and black people, (who also seem to be more severely aected). This disease is the
paradigm of heart failure with systolic dysfunction, with all the signs
and symptoms of that case.
Treatment for this condition is the same as that of heart failure with
depressed systolic function (Chapter 4).
Peripartum cardiomyopathy and Chagas disease may manifest with
clinical evidence of dilated cardiomyopathy. Anthracyclines (adriamycin, doxorubicin, etc.) may cause dilated cardiomyopathy, thus it is important to reevaluate the systolic function with an echocardiography or
a radionuclide ventriculography before, during and after chemotherapy
cycles.
5.2. Hypertrophic
Cardiomyopathy
Complementary tests reveal cavity growth and systolic dysfunction (Figure 41).
Defined by an increase in ventricular wall thickness in the absence of
abnormal working circumstances (such as high blood pressure or heart
valve diseases) sucient to cause the anomaly (Figure 43).
Figure 41. Apical Four Chamber Echocardiogram view of left ventricle

dilation and loss of normal ventricular geometry in patient with dilated
cardiomyopathy
Cardiac magnetic resonance reveals a pattern of predominantly subepicardial fibrosis, unlike infarct, which usually dominates the subendocardium
(Figure 42).
Figure 43. Layout of hypertrophic cardiomyopathy with septal
predominant
The most important pathophysiologic alteration is diastolic dysfunction

causing structural changes to the myocardium. It increases left filling
pressures and explains pulmonary congestion and its most frequent
symptom: dyspnea.
Increased oxygen demand and anomalies in associated intramural coronary arteries, among other reasons, result in predisposition to ischemia. Approximately 5% to 10% show late progress towards thinning
and myocardial dilation imitating dilated cardiomyopathy.
Figure 42. Four chamber cardiac magnetic resonance imaging in

diastole, revealing dilation of the left ventricle (A) and image obtained
with correct sequence that shows the absence of delayed myocardial
enhancement areas (B). Taken from G. Fernandez, G. Robles, Lopez
Zamorano. Manual de Imagen Cardaca. CTO Editorial, 2010
Septal hypertrophy may result in flow acceleration of the left ventricle outflow tract causing a Venturi eect which, associated with abnormal location of mitral valve papillary muscles, forces the valves into systolic anterior
motions (SAM) and to move towards the septum. This causes dynamic left
ventricular outflow tract obstruction, which occurs at rest in one-third of
patients. Another third of patients does not present obstruction at rest,
29
but does with Valsalva-type maneuvers, and the

remaining third does not present any significant
dynamic obstruction (> 30 mmHg). SAM is not
pathognomonic of hypertrophic cardiomyopathy.
At ultrastructural level, in addition to hypertrophy,
there are variable degrees of fibrosis and disorganization of myocardial histology adopting a disarrayed pattern, as well as changes to intramural
coronary arteries that show wall thickening and
facilitate the appearance of myocardial ischemia.
Increases murmur
Ventricle contractility
(contraction with more strength)
Preload (less volume to send

with more strength)
Exercise
Isoprenaline
Digital
Calcium antagonists
-blockers
Volume expansion
Venous return
Raised legs
Contractility
Valsava
Sudden standing
Tachycardia
Nitroglycerin
Preload
Afterload
(less flow resistance)
Arterial vasodilators
Exercise
Squatting
Phenylephrine
Afterload
At macroscopic level, it is common to find significant asymmetric hypertrophy (> 15 mm), which
Decreases murmur
is septal in most cases. However, there are other
hypertrophy patterns, such as apical hypertrophic
Figure 45. Murmur variations in hypertrophic cardiomyopathy
cardiomyopathy (Yamaguchis disease, frequent
in Japan), with giant inverted T-waves in the precordial lead (Figure 44) or Likewise, an initially intense, bisferiens pulse is usually present and, if the
obstruction is severe, there is a double inverted splitting of the second
cases of concentric hypertrophy.
heart sound.
Anything decreasing preload (Valsalva maneuver, sudden standing, arrhythmias) and/or afterload (vasodilators, exercise) or increasing contractility (positive inotropes, physical exercise), increases the outflow
tract obstructive gradient and the murmurs intensity. On the contrary,
the murmurs intensity decreases with increased preload (squatting,
raising lower limbs, and volemia expansion) and/or afterload (vasoconstrictors such as phenylephrine, squatting) (Figure 45).
The ECG usually shows certain changes, such as varying degrees of left ventricular hypertrophy and left atrial growth. Abnormal Q waves in the left
precordial lead are characteristic in the absence of infarction. In apical form,
inverted giant T waves may be noted in the precordial lead (Figure 46).
Figure 44. Echocardiographic image (apical four-chamber view) with

echo-enhancer (an increased echogenicity may be noted inside the
heart chambers because of contrast) of a patient with hypertrophic
cardiomyopathy with apical predominance (arrows)
Most patients remain asymptomatic or have mild symptoms. The most frequent symptom is dyspnea, followed by angina. Fatigue, lightheadedness
and syncope (because of various mechanisms, from neural mediation to
other severe, caused by left ventricular outflow tract obstruction or ventricular arrhythmias) are other symptoms. Very frequently symptoms are
heightened by exercise or during postprandial periods. Atrial fibrillation is
frequent during clinical course and usually results in a significant worsening
of symptoms.
Examination may be normal except for the presence of a fourth sound
and, occasionally, a double, prominent apical impulse.
In patients with left ventricular outflow tract obstruction a hard systolic murmur is heard, located in the apex and left sternal border, which may irradiate
to the hearts base but not to the carotids (unlike aortic valve murmur).
30
Figure 46. 12-lead ECG of a patient with hypertrophic cardiomyopathy

with apical predominance. Note they are in atrial brillation, the elevated
voltages of QRS complex in precordial leads and giant inverted T-waves in
left precordial leads.
Heart shape on chest x-ray is usually normal or with mild cardiomegaly,
especially at the expense of the left atrium when it is dilated.
Card iology
Figure 47. Echocardiographic image in parasternal long axis (A) and short axis (B) of a patient with asymmetric septal hypertrophic cardiomyopathy,
showing excessive thickness of intraventricular septum (arrows)
An echocardiogram is the most important study, since it determines

the magnitude and distribution of ventricular hypertrophy (normal
septum diastolic thickness in adults is lower than 12 mm), the presence
of SAM (which enhaces dynamic obstruction), and enables quantitation of basal outflow tract gradient and after Valsalva-type maneuvers;
a frosted appearance of the ventricular septum is a common feature
(Figure 47).
Treatment of diastolic heart failure. Beta blockers are the treatment of

choice due to their capability to decrease contractility and cause bradycardia (then lengthening diastole). However, it is not proven that
-blockers lower the risk of sudden death.
Enhancement (increase) of post-extrasystole gradient and murmur is

very frequent. In apical forms, ventriculography has the morphology of
an ace-of-spades.
Diuretics should be used with caution to avoid excessive preload decrease which would worsen the obstructive gradient; they are useful for
relieving pulmonary congestion.
Dierential diagnosis must be conducted on children with multisystemic

syndromes and ventricular hypertrophy (Noonan, Danon syndrome, deposit or metabolic diseases) and on adults, especially with aortic stenosis, hypertension and physiologic hypertrophy because of exercise that
occurs in athletes. The latter may be complex sometimes; it is useful
to apply analysis techniques of regional diastolic functions by means of
echocardiography, cardiovascular magnetic resonance or temporarily
suspending sport activity, which tend to modify the hypertrophy pattern.
In the case of supraventricular or ventricular arrhythmias, the anti-arrhythmic

drug of choice is amiodarone, since in addition to its anti-arrhythmic potential it has a negative inotrope and anti-ischemic eect.
Most patients remain asymptomatic or have mild symptoms. However,

there are other possible courses for the disease which provide for a
poor prognosis, such as heart failure (usually diastolic), left ventricle
outflow tract obstruction, development of atrial fibrillation associated
with a significant increase of thromboembolic complications, and the
presence of ventricular arrhythmias, which can regrettably make the
disease result in sudden death. In fact, in all likelihood, hypertrophic
cardiomiopathy is the most frequent sudden death cause of cardiac origin in young people and competition athletes.
Progress towards dilated forms in the long term is infrequent (5% to
10%). Pregnancy is usually well tolerated.
Annual mortality is approximately 1%. The most frequent cause is sudden death, resulting from ventricular arrhythmias, usually polymorphic,
with a higher risk among children (up to 6% annually) who also present
major hemodynamic mechanisms in addition to arrhythmia.
Treatment includes the following:
Eective alternatives (in case of contraindications or inecacy) are disopyramide, verapamil or diltiazem. In refractory cases they may even be combined.
Disopyramide or sotalol may be an alternative.

Preventive treatment with anti-arrhythmic drugs to decrease risk of sudden death is not prescribed. Atrial fibrillation, in addition to worsening
symptoms when it appears in patients, involves high stroke risks and
points to the need for chronic oral anticoagulants. Sinus rhythm may be
hard to maintain in the long term, both by anti-arrhythmic drugs (those
in the Ic group are contraindicated) and by catheter ablation procedures.
There is a series of factors that enable identifying patients with higher
sudden death risk (Table 21).
HIGH RISK FACTORS

Aborted sudden death
Spontaneous sustained
monomorphic VT (SMVT)
Premature sudden death event in
first degree relatives
Recent syncope of unknown origin
Septal thickness > 30 mm
Nonsustained Holter VT (especially
among young people)
HBP during ergometry
POTENTIAL RISK MODIFIERS

Obstructive gradient
Unfavorable genotypes
Delayed enhancement
gadolinium areas
in myocardium as a result
of fibrosis
Left ventricle apical aneurism
Table 21. Factors related to sudden death risk in hypertrophic

cardiomyopathy
31
Besides aborted sudden death or spontaneous SMVT (the presence of

which suggests implanting an ICD as secondary prevention), remaining sudden death risk factors have a low positive predictive value (10%
to 20%) and a reasonable negative predictive value (85% to 95%). For
these reasons, there is dissent about implanting an ICD in the presence
of one or more risk factors.
In patients showing significant obstructive gradient with relevant
symptoms, beta blockers (calcium antagonists in the alternative) are
the first line of treatment, with the possibility of adding disopyramide
in refractory cases.
If in spite of optimum medical treatment, a severe obstruction gradient
persists (> 50 mmHg at rest) together with disabling symptoms (usually
NYHA III-IV class), an intervention may be considered to decrease or
alleviate said gradient:
Septal myectomy (resection of part of the hypertrophied septum),
with or without intervention on the associated mitral valve if applicable, has been shown to improve symptoms and mitral insuciency in the long term. If surgical risks are not disproportionate, it
is probably the alternative of choice when relying on expert hands.
A therapeutic alternative is alcohol septal ablation, which causes a
nonsurgical septum reduction by means of cardiac catheterization
and causing a controlled septal infarction, by infusing ethanol to the
septal coronary branch. It requires favorable septal branch anatomy
and extensive experience. A significant ratio (5% to 25%) of patients
suer complete AV block after the procedure and require permanent stimulation with pacemaker, and there is some concern about
the risk of late ventricular arrhythmias due to the scar created, especially if there is residuary gradient.
Implanting a dual chamber pacemaker (DDD) changes the ventricular depolarization sequence, triggering gradient decrease and
hemodynamic and symptomatic improvement in some patients
(especially elderly patients). There is dissent about its real ecacy,
especially in the long term. However, it is more available and has
fewer complications than septal reduction techniques.
increase resistance to filling and cause symptoms to progress. Atrial fibrillation is very frequent, and up to one-third of patients suer stroke
episodes.
Further, physical examination is similar to what may be seen in constrictive pericarditis, with increased venous pressure, Kussmaul sign, decreased sound intensity, third or fourth extra sounds. A deep and quick y
descent wave predominates in the jugular venous pulse, an increased a
wave, of similar width to v, and an x descent wave that can also be quick
adopting a W morphology. Apical impulse is easily felt in restrictive
cardiomyopathy even if it is decreased, which does not occur in constrictive pericarditis.
Table 22 shows the main dierential diagnoses of restrictive cardiomyopathy.
POSSIBLE RISK FACTORS

IN SPECIFIC CASES
HIGH RISK FACTORS
Recovery from sudden death

Spontaneous sustained
monomorphic VT
Family history of premature
sudden death
Syncope of unknown origin
(especially in children)
Low blood pressure in
ergometry
Septal thickness > 30 mm
Nonsustained VT on Holter
Atrial fibrillation
Myocardial ischemia
Obstructive gradient
Unfavorable genotypes
Intense physical exercise
Under 30 years of age at
diagnosis
Table 22. Dierential diagnosis of restrictive cardiomyopathy

Treatment is basically symptomatic. Diuretics may alleviate congestive
symptoms. Beta blockers and calcium antagonists may improve diastolic function by diminishing heart rate, although their negative inotrope
eect may worsen the condition in some cases. It is key to detect potentially treatable secondary causes.
Loef ler Eosinophilic

5.3. Restrictive Cardiomyopathy Endomyocardial Disease
This is characterized by the presence of restrictive physiology for ventricular filling (with small increases in volume there are large increases in intraventricular pressure, due to increased rigidity of ventricular walls) in
the absence of significant dilation or abnormal parietal thickening of the
ventricles. It is much less frequent than dilated and hypertrophic cardiomyopathy (in tropical Africa it comprises up to 25% of heart failure cases).
In this condition, just as in constrictive pericarditis (with which it is
necessary to conduct a dierential diagnosis, since it may benefit from
surgical treatment), the initial part of diastole is not compromised, drastically reducing ventricular distensibility when it reaches its relaxation
capability limit. On the contrary, with cardiac tamponade all the diastole is involved.
It is similar to constrictive pericarditis, with exertion dyspnea, fatigue,
and predominant systemic venous congestion (hepatomegaly, ascites). Obliteration of the cavity by thrombus or progressive fibrosis
(typical in apical segments, especially in endomyocardial fibrosis) may
32
Significant (over 1,500 eosinophils/mm3), persistent (hypereosinophilic syndrome) eosinophilia may aect multiple organs (lungs, bone marrow, brain)
and especially the heart, where Loeers eosinophilic endocarditis with endomyocardial fibrosis may appear, key to determine the diseases prognosis.
In most cases it occurs in temperate countries and the cause is unknown.
Clinical features are the result of venous and pulmonary congestion secondary to impaired ventricle filling. The posterobasal region of the left
ventricle is frequently aected, involving the posterior leaflet and with
mitral valve regurgitation. Treatment is for diastolic heart failure and anticoagulation, corticosteroids and hydroxyurea or interferon may be added.
Surgery may obtain good results in certain cases with established fibrosis.
Endomyocardial Fibrosis
or Davies Disease
There is endomyocardial fibrosis, mainly in both ventricular outflow
tracts and in the right ventricular apex, leading to obliteration of cavi-
Card iology
ties and, frequently, aecting valves. It manifests mostly in children and

young adults living in tropical and subtropical areas in Africa. It may be
accompanied by intense eosinophilia, but not in all cases. Heart valve
diseases symptoms added to the clinical features of restrictive cardiomyopathy.
When medical treatment of heart failure is unsatisfactory, and in advanced cases, surgical resection of fibrous endocardium replacing affected valves is the alternative of choice.
Amyloidosis
It is rare for secondary amyloidosis (systemic amyloidosis [AA]) to affect the myocardium, while cardiomyopathy is the most frequent cause
of death in primary amyloidosis. Here (systemic amyloidosis [AL]), the
heart is involved in up to 90% of cases. It may be caused by any hematologic disease with clonal expansion of B-cells, since the protein that
is deposited is immunoglobulin light chain, and in up to 80% of cases
it occurs in relatively benign gammopathies. Organs other than the
heart are frequently aected. Transthyretin-related (TTR) amyloidosis
may cause familiar cases of heart amyloidosis.
Amyloidosis may cause restrictive cardiomyopathy (most frequently),
dilated cardiomyopathy in advanced stages, in some cases ventricular
hypertrophy (infrequent in other etiologies of restrictive cardiomyopathy), atrial or ventricular arrhythmias, conduction disorders or orthostatic hypotension. Sudden death is not exceptional. This disorder
shows a preference for right cavities, at least in early stages. Pericardial
eusion is very frequent.
A characteristic echocardiographic feature is a shiny speckled appearance of the myocardium with thickening of intraventricular septum.
Treatment for plasma cell dyscrasias may be eective on patients
during initial stages of the disease, although the general prognosis is
bleak.
Focal amyloid depositions frequently appearing in the heart of elderly
people (senile amyloidosis) lack clinical relevance.
Endocardial Fibroelastosis
This is a disease typical of fetus and infants, of unknown etiology, although it appears to be related to certain virus such as parotitis and
autoimmune phenomena, involving elastin and collagen depositions in
the endocardium, quickly progressing from a picture of initial restriction
to dilation with a very poor prognosis.
Hemochromatosis
This disorder should be suspected in patients with signs of heart failure associated with alterations of liver function, diabetes and increased
skin pigmentation. Cardiac damage may also occur in the nongenetic
secondary forms because of increased iron supply (e.g., from multiple
transfusions).
The clinical picture is usually a mix of systolic and diastolic dysfunction
with frequent associated arrhythmias. Iron depositions are predominant in the subepicardium. Repeated bloodletting and deferoxamine
may be helpful.
Anderson-Fabry Disease (diffuse

angiokeratoma)
This is a recessive inherited disorder, linked to the X chromosome, due
to a deficiency of lysosomal enzyme alpha-galactosidase A. There is intracellular buildup of glycosphingolipids aects multiple organs, mostly
skin, kidneys and myocardium, frequently involving the mitral valve.
Endothelial cell deposits causes occlusion of small arteries, potentially
developing angina or infarction even with normal epicardial coronary
arteries. Frequently, the deposit results in myocardial thickening with
an appearance resembling that of hypertrophic cardiomyopathy. Systolic function is usually normal or near normal, but diastolic failure is
the rule, although it is not usually severe. Enzyme replacement therapy
results in improved symptoms and prognosis.
5.4. Other Cardiomyopathies

Right Ventricular Arrhythmogenic
Cardiomyopathy (ARVC)
This is characterized by gradual replacement of normal myocardial tissue
with fibrofatty tissue, predominantly in the region known as triangle of
dysplasia (i.e., right ventricular outflow tract, apex, and inflow tract),
although it may extend to the rest of the right ventricle and even to the
left ventricle (mainly posterolateral wall). The clinical eect is the formation of intramyocardial reentrant circuits in the fibrofatty replacement
area which facilitates the development of sustained monomorphic ventricular tachycardia, typically during exertion, even before developing
morphologic abnormalities that may be detected, presumably making
this cardiomyopathy the main cause of sudden death in sportspersons of
certain European regions. In advanced stages, right ventricle impairment
progress towards severe right-sided heart failure; when both ventricles
are aected it may mimic a dilated cardiomyopathy.
Its prevalence is 1:5,000. Recessive autosomal patterns have been reported (Naxos and Carvajal diseases are caused by mutations in plakoglobin and desmoplakin coding genes, respectively), but most cases are
caused by dominant autosomal inheritance due to mutations to plakophilin-2 genes and other desmosomal proteins.
Diagnosis is based on the presence of a number of higher and lower criteria in several categories: structural/functional alterations of the right
ventricle, tissue characterization of the same, ECG conduction or repolarization alterations, arrhythmias, family background). Such criterion
provides the basis for a conclusive diagnosis, to detect borderline or
potential cases.
Incipient cases frequently pose a challenge, even more when its clinical
debut may be an episode of sudden death.
Magnetic resonance is the most sensitive imaging study to detect
structural abnormalities (aneurysms, dyskinetic and akinetic segments in the right ventricle wall and cavity dilation) and intramyocardial fibrosis (with late gadolinium-enhanced technique). Echocardiography and ventriculography with contrast are also useful.
33
Endomyocardial biopsy, which is not usually necessary, confirms fibrofatty degeneration even when the segmented nature of the disease may result in false negatives.
An ECG is very useful even though it may be normal or near normal during initial stages. The most frequent anomaly is the presence of inverted
T-waves in right precordial leads (V1-V3) in people over 14 years of age
(prior to this, they may be normal) in the absence of right bundle branch
block. The most specific abnormality is the presence of epsilon waves
in V1-V3 (signs of low voltage in the final part of QRS, which is usually
slightly widened, a reflection of late activity of involved regions). Low
voltage is also frequent (Figure 48).
currence avoidance cannot be guaranteed. ICD is prescribed for sudden death survivors and high risk individuals (such as those showing
impaired left ventricle and those with ventricular tachycardia, especially
if it is recurrent in spite of anti-arrhythmic drug therapy and/or catheter
ablation).
Treatment of right-sided heart failure (diuretics, water deprivation) is
applied in advanced cases.
Spongiform Cardiomyopathy
(noncompacted myocardial)
There is a developmental defect in the growth of left ventricle myocardial wall sinusoids, which is therefore highly trabeculated and with large
intertrabecular recesses, resulting in a ventricular wall with spongy appearance. Patients may progress towards dilated forms, and the first
episodes may be strokes or arrhythmia. Very frequently it runs in the
family, with several involved mutations that have been reported. It may
occur both in isolation as well as accompanying complex congenital
heart diseases, Ebsteins anomaly, or neuromuscular disorders.
Tako-tsubo Cardiomyopathy (dyskinesia

or transient apical ballooning)
Initially reported in Japan, it usually aects postmenopausal women following an emotionally or physical stressful situation in 65% of cases (death of a
relative, medical procedures), and causes chest pain and electrical changes
(ST-segment transient elevation followed by profound, diuse T-wave inversion) similar to an acute myocardial infarction in the absence of coronary
disease, together with mid-ventricular systolic and/or apical dysfunction, disproportionate to the slightly increased cardiac enzymes reflecting myocardial injury and over a surface greater than a single blood vessel (Figure 49).
Figure 48. 12-derivation ECG of a patient with right ventricle dysplasia,

where waves (epsilon) (arrows) and inverted t waves may be noted in
right precordials V1-V4
A documented ventricular tachycardia episode with left bundle

branch block morfology (since it starts in the right ventricle) and
upper axis strongly supports its diagnosis.
First-degree family confirmed history of disease, verified presence
of one of the mutations responsible or even a sudden death family
event attributable to this disease also strongly support this diagnosis.
Treatment is basically symptomatic. Sustained monomorphic ventricular tachycardia episodes are treated with electrical cardioversion and/or
anti-arrthymic drugs if they are well tolerated or recurrent. Beta blockers, sotalol and amiodarone are eective for prevention.
Tachycardia reentrant circuit catheter ablation involves a limitation,
since potential circuits are multiple and progressive, and therefore re-
34
Figure 49. Contrast ventriculography revealing dilation and dyskinesia

(ballooning) of left ventricular apical region in a female patient with
tako-tsubo cardiomyopathy
Card iology
Although its origin is unknown, it has been related to surges in sympathetic activity (stress cardiomyopathy) and usually returns within the first two
months. Treatment is empiric and customized, usually applying the treatment for acute coronary syndrome with ventricular dysfunction (beta blockers, ACE inhibitors). Prognosis is usually favorable and recurrences are rare.
ISCHEMIC HEART DISEASE

Chronic coronary syndromes
Chronic stable angina

Microvascular angina (X syndrome)
Silent ischemia
Acute coronary syndromes
With a persistent elevation of the

ST: transmural acute myocardial
infarction (with Q wave)
Without a persistent elevation
of the ST: subendocardial infarction
(without Q wave), unstable angina,
Prinzmetals angina
Coronary Artery
Chapter 06
Disease
Heart failure
Ventricular arrhythmia and sudden death
Table 23. Clinical forms of ischemic heart disease
6.1. Concepts
Coronary embolisms, ascending aortic aneurysms, when proximally

dissected, or congenital abnormalities of the coronary anatomy.
Demand increase because of myocardial hypertrophy: hypertensive heart disease, aortic stenosis, hypertrophic cardiomyopathy, or
tachycardia.
Reduction of oxygen contribution because of anemia, increased carboxyhemoglobinemia or other conditions.
Figure 50 shows a diagram of the coronary anatomy.
Aorta
Left main coronary

artery
Stages of Coronary Atherosclerosis
Circumflex artery
Right coronary
artery
Atherothrombosis is a chronic inflammatory disease which commences with an endothelial dysfunction facilitating the flow of LDL cholesterol towards the subendothelial space, which is then oxidized and
esterified, and seeks to be phagocytized by macrophages obtained
from the bloodstream, but these are unable to fully digest such cholesterol and turn it into foam cells which finally commence their own
apoptosis.
Left anterior
descending
artery
Inferior wall
of the right
ventricle
Anterior wall, septum

and lateral wall
Posterior,
lateral
and inferior
wall
His bundle
55%
90%
Sinoatrial node
AV Node
45%
10%
Figure 50. Cardiac revascularization

The concept of ischemic heart disease encompasses all myocardial alterations resulting from an imbalance between the supplies and the demand of oxygen, with impaired cardiac function. It can manifest itself in
dierent ways, as shown in Table 23.
The etiology of ischemic heart disease is usually atherosclerosis of the
epicardial arteries. Other causes may include:
Alterations of the coronary microcirculation (microvascular angina
or syndrome X), endothelial dysfunction, etc.
Coronary spasm (variant, vasospastic or Prinzmetal angina) generally occurs in areas close to small atheroma plaques, but can also be
triggered by cocaine, ergotamine or other vasoconstrictors.
The release of cytokines attracts other inflammatory cells (monocytesmacrophages and lymphocytes), which multiply the release of cytokines and, among other phenomena, increase even more the endothelial permeability and dysfunction. Smooth muscle cells migrate towards
the subendothelial space and synthesize collagen, which attempts to
stabilize the increasing atheroma plaque with a higher or lower eciency, since the inflammatory cells produce enzymes that degrade the
matrix (metalloproteinase) and tend to destabilize it.
This generates the atheroma plaque, with a lipid core -formed by LDL
cholesterol esters- which is sometimes even crystalized, surrounded by
inflammatory cells, smooth muscle and collagen in dierent proportions, with the presence of vulnerable plaques (high contents of lipids
and inflammatory cells) and stable plaques (high fiber contents; Figure
51).
The presence of ruptures in the vulnerable plaques exposes the subendothelial material to the bloodstream and activates the platelets, which
attach and aggregate themselves, and set in motion the coagulation
cascade, thus producing the thrombosis of the atheroma plaque which
triggers the acute coronary syndromes (ACS), so that, if the occlusion is
complete, it produces an ACS with a persistent ST segment elevation in
the ECG (which causes a transmural infarction), and if the occlusion is
subocclusive, it originates an ACS without ST segment elevation (with
dierent variables ranging from the subendocardial infarction to the
unstable angina; Figure 52).
35
Figure 51. Development of atherosclerosis
Figure 53. Plaque complication risks depending on the luminal stenosis

caused by plaque growth
As an approximation, when a plaque occludes 70% of the arterial lumen, it produces an ischemia triggered by physical exertion, cold or
emotional stress, but not at rest. When the stenosis exceeds a value
of 80% to 90% (serious or severe), it can produce an ischemia at rest.
The main risk marker for cardiovascular diseases is age. Another important
marker is male sex. No action whatsoever is possible on these two factors,
i.e., they are nonmodifiable risk factors. The increase in life expectancy is,
therefore, one of the elements which has influenced a higher incidence and
prevalence of ischemic heart disease among the population.
Smoking, hypercholesterolemia (total cholesterol above 200-220 mg/
dL, LDL cholesterol above 160 mg/dL and/or HDL cholesterol below
35 mg/dL), hypertension, and diabetes mellitus are undisputed risk factors for ischemic heart disease (Table 23). An increased carotid intimamedia thickness, detected by means of a sonogram, is an important
marker of cardiovascular prognosis, despite the fact that its use as a
therapeutic target has not been broadly validated.
Recent studies on the eciency of various measures used in preventive
cardiology reveal that the prevalence of high cholesterol values is decreasing significantly. However, smoking or hypertension values experienced
no material modification, the prevalence of diabetes has increased, and
the prevalence of obesity has drastically increased (Table 24).
Figure 52. Pathophysiology of acute coronary syndromes

Generally, the risk of a plaque presenting a complication increases in
proportion to the size of the plaque, even though possible complications also depend on other factors.
Nevertheless, given that the number of small plaques is much greater
than the number of large plaques, it is the small but vulnerable plaques
that generally cause, in absolute terms, an acute coronary syndrome
(Figure 53).
36
RISK FACTORS
Male sex
Hypertension
Tobacco consumption, especially cigarettes
Obesity, body mass index 30 kg/m2
Sedentary lifestyle
Dyslipidemia, high LDL cholesterol or low HDL cholesterol
Diabetes
Microalbuminuria
Estimated glomerular filtration < 60 mL/min
> 55 years of age in males and > 65 years of age in females
Family history of premature cardiovascular disease in men under 55
years of age and women under 65 years of age
Table 24. Main cardiovascular risk factors
Card iology
The eects of ischemia on the myocardium are summarized in Figure

54.
Perfusion
(with profusion)
initial event) imposes a marked limitation on the patients habitual activities is also deemed unstable (Table 27).
Nonrecoverable
AORTIC DISSECTION
Exercise
(dobutamine,
dipyridamole)
SPECT
Gammagraphy
(Tc, TI)
Stress echo
Healthy
Stunned Hibernating
myocardium myocardium myocardium Necrosis
Acute ischemia Chronic ischemia
No defects
Reversible defects
Irreversible defects
Rest
The nature and location of the pain may be

similar to that of the coronary, but this one
is prolonged, usually pleuritic, and changes
with the patients posture (it is alleviated by
ACUTE PERICARDITIS
flexing the trunk). Pericardial rub. Concave and
diffuse elevation of the ST segment. It can be
alleviated with anti-inflammatories and not
with nitroglycerin.
AORTIC STENOSIS
Exercise angina. Exercise syncope. Dyspnea.

Aortic systolic murmur irradiated to carotid.
MITRAL PROLAPSE
Usually atypical pain, with very variable duration

and no clear triggering factors, which cannot be
alleviated with nitroglycerin. Auscultation of a
mid-systolic or end-systolic click.
PULMONARY
HYPERTENSION
It is originated by a right ventricular ischemia.

It may be very similar to an angina and is
connected to acute pulmonary embolism or
chronic pulmonary hypertension events.
ESOPHAGEAL SPASM
Epigastric and retrosternal pain. Usually

connected with the intake of food, especially
when they are very hot or very cold. Like
angina, it can be alleviated with nitroglycerin.
It can cause dysphagia.
Figure 54. Eects of myocardial ischemia
6.2. Angina Pectoris

Angina is the clinical translation of a transitory myocardial ischemia,
most frequently caused by coronary atherosclerosis. The typical patient
is a male over 50 years old, with cardiovascular risk factors presenting, following physical exertion of a given intensity or an episode of
emotional stress, a retrosternal oppression which commences gradually and disappears progressively with rest or sublingual nitroglycerine.
Episodes generally last less than 10 minutes. When only two of these
three characteristics are present, the event is said to be an (probable)
atypical angina.
This oppression or heavy feeling can be irradiated to other zones (upper
limbs up to the fingers, precordium, jaws or teeth, torso, etc.) and be
accompanied by other symptoms such as dyspnea or a persistent vegetative state (cold sweats, anxiety, nausea, asthenia, imminent death
sensation, etc.).
Burning epigastric and retrosternal pain

GASTROESOPHAGEAL manifesting itself especially when lying down
REFLUX
after meals. Presence of acidity in the mouth.
Quickly alleviated with alkaline substances.
PEPTIC ULCER
Epigastric pain. It is worsened by fasting and

alleviated by ingesting foods and taking
antacids.
BILIARY DISEASE
It is localized to the right hypochondrium,

although it can be irradiated to the right
and epigastric hemithorax. It is a prolonged
pain with colic characteristics (it comes
and goes), and responds to analgesicsanticonvulsant medication.
PANCREATITIS
Intense epigastric pain irradiated towards the

back as a belt. It decreases when the patient
bends forward.
Table 25 lists the characteristics of the dierent causes of thoracic

pain.
The most characteristic element of the stable angina is that its clinical
signs always appear, in all patients, after a similarly intense eort. The
classification created by the Canadian Cardiovascular Society (CCS) is
used to establish such eort levels (Table 26).
OSTEOMUSCULAR
PAIN
Sometimes, the angina follows a circadian pattern in which the eort

threshold is lower in the mornings, the postprandial period or with cold.
Women are more likely to present atypical symptoms.
PSYCHOGENIC PAIN
When a patient with stable angina shows symptoms at rest or for prolonged periods of time (up to 20 minutes), or more intense symptoms
with progressively lower eorts throughout a four-week period, the angina is said to have destabilized and is known as an unstable angina.
An angina which, having commenced recently (two months after the
The pain is sudden, persistent and

especially intense (transfixing, tear) from
the very beginning. It migrates to the
zones to which the dissection expands.
Asymmetric reduction of arterial beats. Aortic
insufficiency murmur. Mediastinal widening
in chest x-rays.
Pain in the surface of the thoracic wall

reproduced with mechanical palpation and
exacerbated when moving or coughing.
Dull and persistent precordial pain with acute
pain crisis lasting a couple of seconds. It is
triggered by anxiety and family, economic and
self-esteem troubles. It is in no way connected
to physical exertion. It usually causes
dyspnea, hyperventilation, palpitations, sighs,
paresthesia, and general weakness. It can
be alleviated by very different means: rest,
exercise, tranquilizers, analgesics or placebo.
Table 25. Dierential diagnosis of chest pain
37
Angina only during strenuous efforts. Does not limit ordinary

lifestyle.
II
Slight limitation of physical activity. The angina manifests

itself when walking fast or climbing stairs or steep roads.
Patients can walk more than one or two blocks or climb one
flight of stairs.
to be 2% to 3%, even though recent studies show it to be lower (not

exceeding an annual 1%).
ANGINA AT REST
III
Strong limitation of physical activity. The angina manifests itself

after walking one or two blocks or climbing one flight of stairs.
IV
Inability to perform any activity without angina. This

symptom can manifest itself while at rest.
Table 26. Clinical seriousness of angina. Classication by the Canadian
ANGINA OF
RECENT ONSET
Recently commenced angina ( > 2 months)

reaching at least Class III on the CCS classification
ACCELERATED
ANGINA
In the previous four weeks, increase in the

number, intensity, duration or reduction of the
threshold of onset in a patient suffering from
stable stress angina
Cardiovascular Society
The general treatment of a patient with stable angina is shown in Figure 55.
The diagnosis of chest angina is based on the patients medical history,
with its logic and limited sensitivity, and is complemented with additional tests, such as a baseline ECG and an ergometry.
With current treatment, the prognosis of patients with stable angina is
usually favorable. The historic annual mortality rate has been estimated
Angina starting at rest. It usually persists for a

prolonged period of time (> 20 minutes)
POST-INFARCTION Angina manifesting itself in the first few days

following a myocardial infarction
ANGINA
Table 27. Classication of unstable angina

There are two ways to detect ischemia; exercise stress testing (assessing repolarization alterations during physical exercise) and imaging techniques (stress echocardiography or nuclear cardiology).
Suspected angina
Unstable angina
ACS management
Anamnesis
Physical exploration
ECG at rest
Lab tests
Chest XR, if applicable
Alternative diagnosis
Confirm and treat
Assess ischemia
Of choice: exercise testing
Stress test imaging procedure when:
- Inability to exercise
- Difficulties in reading ECG
- Inconclusive ergometric data
No
YES
Assessment of risk
Calculate LVEF
Low
Medical
treatment
Good
Medium
High
General measures
ASA or clopidogrel
Statin (goal LDL < 100 or < 70 in high-risk patients)
ACE inhibitor with confirmed cardiovascular disease (consider in all cases)
-blockers in patients with AMI or a history of heart failure
Anti-anginal treatment:
- Of choice: -blockers + sublingual nitroglycerin in crisis
- Association or alternative: calcium antagonists, prolonged action nitrates, ivabradine, others
(nicorandil, trimetazidine, ranolazine)
Assessment
of clinical response
Consider
coronarography
Coronarography
Poor
Coronarography
Revascularization, when possible
Figure 55. General treatment of patients with stable angina
38
Card iology
The most physiologic way to induce ischemia, and the one providing
the most information, is physical exertion (exercise stress testing, stress
echocardiography or exercise nuclear cardiology). If the patient cannot
perform physical exercises, a pharmacologic stress must be induced
with dobutamine, adenosine or dipyridamole.
An irreversible perfusion defect in the gammagraphy indicates necrosis

and, if reversible with rest, ischemia.
Ergometries are deemed electrocardiographically positive when the ST

segment displaces at least 1 mm measured at 80 ms from the J point
(end of the QRS complex), Figure 56.
Figure 56. Positive exercise stress testing with consistent alterations

in ST-depression in II, III, aVF, V4-V6, and ST-elevation in aVR
It is diagnostic test when the patient attains 85% of the maximum predicted heart rate. The global sensitivity of the test is 75%, which justifies the existence of false positives (especially in women) and negatives.
There is a series of criteria to identify patients with the worst prognosis
after an ergometry (Table 28).
PARAMETERS ASSOCIATED WITH ADVERSE PROGNOSIS

Inability to complete stage II of the Bruce protocol
Ischemic depression of the ST segment 2 mm before completion
of the second stage of the Bruce protocol or at a frequency of < 130 bpm
Early positivity in the first stage or prolonged duration (more than five
to six minutes) of ST segment depression upon exercise completion
Diffuse ST segment depression in five or more leads of the ECG
Elevation of the ST segment in leads without a Q wave
Depression or flat response in systolic blood pressure after increasing
the stress level
Maximum heart rate achieved with limiting symptoms lower than
120 bpm in the absence of a treatment with -blockers
Table 28. Parameters associated with adverse prognosis

Imaging techniques are more sensitive and precise than conventional
exercise stress testing, and must be oered from the very beginning
where electrocardiographic alterations hamper the identification of
ischemic anomalies (left bundle branch block, Wol-Parkinson-White
syndrome, pacemaker, digitalis-induced ST segment depression, ventricular hypertrophy, etc.).
Figure 57. Percutaneous transluminal coronary angioplasty

The patients prognosis must be established by assessing mainly three
factors: ventricular systolic function, the extension and seriousness of
the ischemia, and the presence of ventricular arrhythmias. When faced
with adverse prognosis data, a coronarography must be carried out as
soon as possible to establish the coronary anatomy and proceed with
revascularization, if possible. Current guidelines for the completion of a
coronarography are shown in Table 29.
CORONARY ANGIOGRAPHY
1. Stable angina with low exercise workload despite medical treatment
2. Poor prognosis data in diagnosis tests
3. In the non ST-segment elevation ACS in medium or high risk cases,
and, where positive results are obtained, in ischemia detection tests
in low risk cases
4. In the context of AMI
- Recurrent ischemia
- Cardiac failure or previously ignored diminished LVEF
- Primary PTCA
- Failed thrombolysis
- Mechanical complications
- Residual ischemia in diagnostic tests
5. Sudden death survivors, unless a definite diagnosis has been
reached other than ischemic heart disease (long QT, Brugada
syndrome, and so forth)
Table 29. General guidelines for a coronary angiography (continued)
39
CORONARY ANGIOGRAPHY
6. Valvular heart disease preoperative period: when there is a risk of
coronary artery disease. Consider CT angiography as an alternative
to an invasive angiography
7. Assessment of the dilated cardiomyopathy to discard ischemic
causes. Consider CT angiography as an alternative to an invasive
angiography
8. Patients with chest pain requiring a certain diagnosis (professional
pilots, drivers). Consider CT angiography as an alternative to an
invasive angiography
Table 29. General guidelines for a coronarography (continued)

The general treatment of stable angina is shown in Table 30.
PROVEN PROGNOSIS
IMPROVEMENT
ASA (or clopidogrel)
Statins
ACE inhibitors where there is a
confirmed cardiovascular disease
-blockers where there is a history
of AMI or ventricular dysfunction
Coronary revascularization if
indicated
SYMPTOMATIC
IMPROVEMENT
-blockers
Calcium antagonists
Nitrates
Ivabradine
Coronary revascularization
Nicorandil
Ranolazine
Trimetazidine
Molsidomine
Allopurinol?
Pain therapy
Table 30. Treatment of stable angina

When required, the usual way to complete a coronary revascularization is
by means of a percutaneous coronary intervention (PCI; see Figure 57).
Surgical revascularization using aortocoronary bypass is used basically
in the left main coronary disease or three-vessel disease with associated ventricular systolic dysfunction, and in a two-vessel disease, where
one of them is the proximal anterior descending artery, especially with
an impaired systolic function (Figure 58).
The main limitations of the PCI are the risk of suering a thrombosis (if
a stent is used in this cases, it extends over time and makes the use of
two stents mandatory, which causes an excessive scarring of the lesion resulting from the angioplasty). They only measure proven useful
to prevent restenosis (usually appearing as a progressive stress angina
three to six months after the CPI) is the use of stents (especially with
the new stents releasing antiproliferative agents). The use of drugeluting stents is recommended together with a double anti-aggregation to reduce the incidence of thrombosis from the stent for at least
six months or one year. On the contrary, when bare metal stents are
used, the use of drugs need only be maintained for one month.
PCI and surgery present similar mortality rates in cases of multivessel
disease, wherefore the disease can be indistinctively approached with
either technique (unless the patient is diabetic, in which case, surgery
will result in a better prognosis), even though more subsequent revascularization procedures are required after a PTCA and cerebrovascular
accident rates are higher after surgery.
The pathophysiology of Prinzmetal -or variant angina is coronary
spasm. The episodes usually occur at rest and preferably at night, and
cause elevation of the ST segment. The recommended treatment is the
use of calcium antagonists and nitrates. The intracoronary infusion of
acetylcholine or ergonovine enables physicians to induce spasms and
confirm the diagnosis.
Syndrome X (demonstrable angina and ischemia in detection tests with
normal epicardial coronary arteries) is usually the result of a dysfunction of the microvascular endothelium in patients with multiple cardiovascular risk factors.
Acute Coronary
Chapter 07
Syndrome
7.1. Acute Coronary Syndrome
Without ST Segment Elevation

Most ACS presents a common pathophysiology, a rupture or erosion phenomenon followed by thrombosis in an atheroma plaque,
together with coronary spasms and distal embolization of thrombus
fragments.
Figure 58. Myocardial revascularization with internal mammary artery

graft and aortocoronary saphenous vein graft
40
The degree of occlusion of the vessel lumen determines whether

there will be an acute coronary syndrome with a persistent rise/elevation (> 20 min) of the ST segment (STE-ACS, usually a complete occlusion and evolving towards a transmural infarction) or an acute coronary syndrome without a persistent rise/elevation of the ST segment
(NSTE-ACS, usually subtotal or intermittent total occlusion because of
distal embolization of thrombus fragments rich in plaques, including
unstable angina). The distal embolization of small thrombus fragments
is suspected to determine the presence of isolated sites of necrosis
Card iology
surrounded by myocardial inflammation zones causing the increase in

troponins (Figure 59).
The incidence of NSTE-ACS is somewhat greater than that of the STE-ACS.
The hospital mortality rates of this second case are higher (7%) than those of
NSTEACS (5%), but after a few months mortality rates become equal for both
cases (around 12% after six months) and even seem to be higher in the case
of NSTE-ACS in the long term, probably because this condition usually aects
older patients with higher comorbidity levels (diabetes, renal failure, among
others) and a longer history of coronary disease. Therefore, recurrent reinfarction and ischemia rates are higher in patients suering NSTE-ACS. The
general steps in case of an NEST-ACS are shown in Figure 60.
Current recommendations suggest that the risk should be assessed (hospital and long-term mortality) using one of the available scales (GRACE, TIMI,
PURSUIT, etc.), which collect clinical variables with prognosis implications
(age, heart rate, blood pressure, Killip class, diabetes, and coronary history),
electrocardiographic, biochemical and imaging findings (mentioned above).
The prognosis factors shown in cases of stable angina are also useful in this
context. These enable the patient to be placed in the low, medium or high
risk groups, and the treatment to be adjusted accordingly (Table 31).
Figure 59. Unstable plaques in atherosclerosis
Suspected NSTE-ACS
STE-ACS
Anamnesis
Physical exploration
ECG at rest (< 10 min) include V3R, V4R, V7-V9
Lab test: troponin, CPK-MB, hemoglobin, leukocytes, creatinine
Alternative diagnosis
Specific management
Appropriate tests and treatment

Confirmed NSTE-ACS
Rest, O2 if PaO2 < 90 mmHg

ECG/ST monitoring
Repeat ECG after 6 and 24 h
Repeat troponin after 6-12 h or after 3 h in ultrasensitive ones
Echocardiography at rest
Estimate hemorrhagic risk
Estimate risk and commence medical treatment
Medium-high risk
Low risk
ASA + clopidogrel or ticagrelor if moderate-high risk

Anticoagulation
Statin (precocious)
Antianginals: -blockers (of choice) + nitroglycerine, others
ACE inhibitor/ARA (in most cases)
No recurrence
No heart failure
No changes in the ECG
No increased troponins
Extrem risk (10%)

If:
Refractory angina
Recurrent angina with generalized ST or T wave changes
Heart failure
Hemodynamic instability
Serious arrhythmias
Medical treatment and...
Coronarography
< 72 h
Urgent
coronarography
Revascularization
Ischemia detection test
Medical
management
Elective coronarography
Upon discharge:
Changes in lifestyle
Medical treatment
CVRF control
Figure 60. Unstable plaques in atherosclerosis
41
CLINICAL
At-rest or prolonged angina

Hemodynamic alterations (heart
failure, mitral insufficiency, peripheral
hypoperfusion)
Old age (> 75 years)
Diabetes mellitus
ELECTROCARDIOGRAPHIC
Changes in the ST segment or profound

T wave inversion in all precordial leads
ECHOCARDIOGRAPHY
LAB TESTS
Ventricular dysfunction
Extended contraction abnormalities
Assessment of markers:
- Necrosis (T or I troponins, CPK-MB)
- Inflammation (C-reactive protein)
- Neurohumoral activation (NT-proBNP, BNP)
Renal impaired renal function
OTHERS
Previous bypass surgery

PTCA over the last months
Post-infarction angina
Table 31. High-risk criteria in patients with unstable angina
7.2. Acute Coronary Syndrome
with ST Segment Elevation

At present, acute myocardial infarction (AMI) is considered upon the
existence of myocardial necrosis on an ischemia clinic episode; that
is, elevation and posterior fall of necrosis markers levels (preferably
troponins with values above the 99.th percentile, which is the normal
baseline) with at least one of the following factors (remember that in
patients with characteristic symptoms with some of the other criteria,
treatment must be commenced despite the fact that markers valuation
may not be still available):
Symptoms compatible with ischemia.
Changes in ECG compatible with acute ischemia (changes in ST or T
wave or new-onset left bundle branch block).
Development of new pathologic Q waves
Onset of new anomalies in segmental contractility or loss of viable
tissue in imaging.
Sudden cardiac death, commonly preceded by symptoms compatible with infarction, associated with changes in ECG or thrombus
image of coronarography or autopsy.
Elevation of necrosis markers in relation to reperfusion therapy/revascularization:
Increase in troponins above five times the normal 99th percentile
after PTCA.
Troponins elevation above ten times the normal 99th percentile
during the myocardial revascularization surgery associated with
Q waves, left bundle branch block, and occlusion of grafts or
new loss of viable tissue.
Evidence of prior myocardial infarction is considered upon:
Development of pathologic Q waves.
Segmental thinning and hypocontractility image in ventricular wall
with no further possible causes present.
42
Findings in pathologic anatomy compatible with cardiac necrosis.

Operatively, initial classification of acute coronary syndromes, according to initial ECG in ACS, with or without persistent ST segment elevation (20 min) (STE-ACS towards NSTE-ASC), is useful because it modifies
the initial therapeutic attitude. So, most STE-ACSs are to develop a classic AMI, whereas NSTE-ACS may develop a subendocardial AMI, a microinfarction episode (low myocardial damage) or an unstable angina,
according to ischemia intensity.
The most frequent cause of AMI is epicardial coronary thrombosis
occurring generally by rupture of the atheromatous plaque, which
produces mild or moderate lumen obstruction in 75% of cases,
but has high lipid and inflammatory content (vulnerable plaque).
A period of many days between the initial rupture and the coronary thrombotic occlusion causing the STE-ACS is frequent. After 15
minutes from the complete coronary occlusion, myocardial necrosis
begins.
Angina pain, oppressive and retrosternal with characteristic radiation
(or equivalent), usually lasts over 20 minutes. It does not completely
respond to rest or nitroglycerine, and is more intense than angina.
Sometimes, this pain is not present or is atypical (patients with diabetes, the elderly or women). It is frequently accompanied by vegetative
symptoms (cold sweating, nausea, vomiting, anxiety and a feeling of
imminent death). It also appears at rest (sometimes, during or after exercising); it is more frequent early in the morning (due to sympathetic
activation and changes in circadian coagulation and platelet activity).
Prior angina frequently occurs.
Other forms include dyspnea, weakness, arrhythmias, systemic embolism, hypotension, or any of the AMI complications when the initial
episode has gone undetected. It must be mentioned that the highest
myocardial infarction mortality occurs during the first two hours after
appearance of symptoms.
No physical sign is pathognomonic of myocardial infarction; however, sympathetic or parasympathetic activity, signs of ventricular
dysfunction, apical systolic murmur related to ischemic mitral insufficiency, and pericardial rub by meta-infarction pericarditis may appear. If jugular vein pressure increases (with Kussmaul sign and/or
paradoxic pulse), right ventricle infarction (febricula may exist during the first week) or cardiac rupture with tamponade must be suspected.
Killip classification (Table 32) on arrival refers to the degree of hemodynamic compromise of a clinically defined patient. The forrester classification (Figure 61) is related to invasive hemodynamic measures, which
require rectification actions (shown in the figure). Both classifications
have a prognostic influence.
No cardiac failure
II
Mild cardiac failure (crackling, S3, pulmonary congestion)
III
Acute pulmonary edema
IV
Cardiogenic shock
Table 32. Killip classication
Card iology
Figure 61. Basic treatment according to the degree

in Forrester classication
Figure 63. Electrocardiographic progress in MI and localization

of electric alterations
ECG is not usually normal even during the first minutes of the AMI.
It is recommended to register additional leads to evaluate the
right ventricle AMI (V3R and V4R) or posterior wall (V7, V8 and
V9), mainly in patients with inferior AMI associated with those two
subtypes.
Changes may aect the following:
T wave (myocardial ischemic image):
Positive-peaked or isoelectric T: subendocardial ischemia.
Negative T: subendocardial or transmural ischemia.
ST segment (myocardial injury current; Figure 62).
Descended ST: subendocardial injury.
Elevated ST: subendocardial injury.
While necrosis is completed, ST segment tends to return to the isoelectric line. Simultaneously, Q waves (and loss of R wave) develop
in the leads in which ST segment occurs.
Inversion of T wave usually persists or normalizes after several
weeks or months. Persistence of ST elevation in leads in which necrosis waves have developed may indicate ventricular aneurysm
or segments with dyskinetic movements (paradoxic). However, in
some exceptional cases, a complete ECG normalization may occur.
T wave alterations generally take up more leads than STs, which, in
turn, take up more leads than Q waves (the ischemia area is greater
than the injury current area, and this is greater than the electric necrosis area).
In regions opposite to where AMI is localized, electrocardiographic
alterations reciprocal (opposite) to those appearing in the leads localizing the AMI are appreciated, although these changes may also
correspond to concomitant ischemia, in other regions, because of
other compromised coronary bundle branch (Figures 64 and 65).
Figure 62. Injury current in ECG

QRS complex. Pathologic Q waves indicate transmural myocardial
necrosis. There are infarctions with Q waves (which are generally
transmural, consequence of STE-ACS) and infarctions without Q
waves (generally limited to subendocardial or nontransmural, consequence of NSTE-ACS). The new-onset left bundle branch block in
the setting of an AMI usually indicates extensive involvement of the
conduction system, and is associated with bigger-sized infarctions
and of worse-prognosis.
ECG progression of an AMI because of complete occlusion of an epicardial coronary artery shows evolutionary alterations that follow a specific pattern (Figure 63).
Figure 64. Anterolateral MI in acute phase
43
MYOCARDIAL NECROSIS CAUSED BY
Ischemia
Severe, acute or chronic cardiac insufficiency
Myocarditis or myopericarditis, complicates endocarditis
Myocardial damage by contusion, catheter ablation,
endomyocardial biopsy or electric cardioversion
Myocardial infarction in aortic dissection, aortic valvulopathy, severe
arrhythmias, hypertension episodes or pulmonary hypertension,
(pulmonary embolism, idiopathic, etc.)
Cardiomyopathy such as hypertrophic, tako-tsubo or infiltrating
Severe episode of sepsis or shock
Cardiotoxic chemotherapy (5-fluorouracil, anthracycline, herceptin)
OTHER CIRCUMSTANCES
Renal function impairement (acute or chronic)

Hypothyroidism
Ischemic or hemorrhagic acute stroke
Large burns or rhabdomyolysis
Some poisoning
Table 33. Causes of cardiac specic troponin elevation
Figure 65. Inferior Wall MI in acute phase

Traditionally, the markers used in the diagnosis of AMI have been creatine phosphokinase (CPK), its CPK-MB fraction, and GOT and LDH enzymes (these two are no longer recommended). At present, the first
choice marker is cardiac specific troponin T or I. The time pattern of the
markers has major diagnostic value (Figure 66).
STE-ACS suspicion
Anamnesis
Exploration
ECG (< 10 min)
SCASEST
V3R, V4R, V7-V9 values
Alternative
diagnosis
Consider ECG
and serial necrosis markers
Relevant
actions
Tests
Treatment
Persistent ST elevation,
left bundle branch block
ECG monitoring
Rest
Oxygen
Anti-emetic opiates
Watch AP and peripheral perfusion
ASA (prasugrel or ticagrelor or clopidogrel
should be added to ASA)
Reperfusion therapy
Yes
Figure 66. Enzymatic evolution in AMI
Primary PTCA possible in less than two hours?
Primary
PTCA
Yes
No
Contraindications?
Thrombolysis
Remember the possible causes of cardiac specific troponin elevation

shown in Table 33.
The initial action recommended in STE-ACS is shown in Figure 67.
The aim of reperfusion therapy is recanalization of the obstructive artery and avoidance of reocclusion. The benefit of reperfusion exists in
the short and long run, and it also minimizes complications and relieves
the pain. Myocardial necrosis is limited when repermeabilizes, reducing
final ventricular dysfunction. This treatment is highly useful in the first
hours after pain has begun. As a general rule, it is indicated in the first
12 hours of clinical course.
No
Consider
coronarography
3-24 h
Effective
Fibrinolysis
(In-hospital
or out-of-hospital)
Ineffective
Rescue
PTCA
Figure 67. Initial performance in STE-ACS

There are two methods of emergency reperfusion: the administration
of intravenous thrombolytic drugs (fibrinolytic medication) or performing emergency coronarography or angioplasty (PTCA or primary PCI).
44
At present, PTCA is considered the first-choice treatment as long as it is

performed immediately (within two hours after consultation) by a spe-
Card iology
cialized team. It has been demonstrated that PTCA provides better clinical
results than in-hospital thrombolysis (less reinfarctions, artery reclusions,
and less residual ventricular dysfunction), and that it may even improve
by using stent, since the risk of the vessel reintervention is reduced.
Door-to-balloon time (the period from hospital arrival to angioplasty)
must not exceed 90 minutes; otherwise, it loses its advantage over fibrinolysis. If the patient presents cardiogenic shock or if fibrinolysis is contraindicated, PTCA is the treatment of choice, regardless of the delay.
Nowadays, in the acute phase, it is recommended to only act over the
artery responsible for the infarction and to consider complete revascularization, when indicated, by PTCA or surgery in a diered way, after
the patient has stabilized.
There follow some contraindications of thrombolytic medication (Table
34) to be checked before their administration to patients.
ABSOLUTE
CONTRAINDICATIONS
Active hemorrhage
(menstruation excluded)
History of intracranial bleeding
Ischemic ictus during the six
previous months
Known brain injury (e.g.,
neoplasia and traumatism)
Severe trauma, major surgery or
head injury in the three previous
weeks
Gastrointestinal bleeding in the
last month
Noncompressible punctures
(lumbar, hepatic biopsy, etc.)
Aortic dissection
Hemorrhagic abnormality
RELATIVE
CONTRAINDICATIONS
Transitory ischemic accident in
the previous six months
Oral anticoagulation
Active peptic ulcer
Pregnancy or immediate
puerperium (one week)
Refractory arterial hypertension
(> 180/110 mmHg)
Severe hepatopathology
Infective endocarditis
Prolonged or traumatic
resuscitation
Apart from the medical treatment, smoking cessation, a cardiac healthy

diet and weight loss are recommended if BMI is greater than 30 and
waist circumference is over 102 cm in men and over 88 cm in women. A
strict control of risk factors, aerobic physical exercising at least five days
a week (including participation in cardiac rehabilitation programs), and
evaluation of arrhythmic risk are also recommended.
In case of requiring a treatment to prevent arrhythmic sudden death, an
implanted cardiac defibrillator is indicated after 40 days (always) from
the occurrence of the infarction. Optimal medical treatment and revascularization must always be granted, if indicated, before the implant.
The main risk determinants are LV ejection fraction and functional class,
as well as nonsustained ventricular tachycardia and inducement of sustained ventricular tachycardia in the electrophysiologic study. It is convenient to wait for the resolution of stunned myocardium in order to
estimate residual ejection fraction. Table 36 shows current indications
to implant a defibrillator after a myocardial infarction.
PRIMARY PREVENTION
(always, at least, 40 days following
the AMI)
LVEF < 40%, VT nonsustained
and VT sustained inducible in
electrophysiologic study
LVEF < 35% and II-III functional
class of NYHA
LVEF < 30% and I functional class
of NYHA (indication IIa*)
SECONDARY PREVENTION
VF-produced sudden death
recovered outside the acute
phase
Sustained VT with poor
hemodynamic tolerance and
depressed LVEF
Sustained VT well tolerated
with normal LVEF (or almost
normal) (indication IIa*)
* Indication shows that the benefit appears greater than the

implantation risk, so it is advisable
Table 36. Indications for automatic debrillator implant after an AMI
Table 34. Contraindications for thrombosis treatment

Rescue PTCA is indicated in the first 12 hours of progression of symptoms
after thrombolysis has failed, and if an increase in ST over 50% is observed
in the leads with the maximum recorded elevation (generally with persistent pain and with no reperfusion arrhythmias). Immediate coronarography
with PTCA is also appropriate upon evidence of recurrent ischemia (post infarction angina) or arterial reocclusion after an initially ecient fibrinolysis.
After the acute phase, medical treatment after a STE-ACS is shown in
Table 35.
LONG TERM TREATMENT IN AMI
Changes in lifestyle
Diabetes control (HbA1c < 7%) and hypertension (AP < 140/90 mmHg)
Acetylsalicylic acid (ASA)
Prasugrel/ticagrelor or clopidogrel for 12 months
Anticoagulation if indicated for any other reason
-blocker
ACE inhibitors and ARAs
Statins
Eplerenone if LVEF < 40% or cardiac insufficiency
Consider ICD and TRS implants
Revascularization if indicated
Table 35. Long term treatment for MI
7.3. Complications
of myocardial infarction
Arrhythmias
Tachyarrhythmias
Mechanisms producing ventricular arrhythmias in the acute phase of
an infarction (primary arrhythmias) dier from those appearing in the
chronic phase of the disease (in which there is an anatomic substrate
for the onset of anatomica re-entries in the scar). The main arrhythmias
are shown in Table 37.
Supraventricular Tachycardia
Sinus tachycardia generally indicates a major infarction with ventricular dysfunction and cardiac insuciency associated. It must not be treated as such.
Sinus bradycardia is common in the acute phase of inferior infarction
due to vagal hypertonicity, or secondary to opiates, and it appears in
25% of cases.
45
ARRHYTHMIA
TREATMENT
SECONDARY PREVENTION
PRIMARY PREVENTION
VF in the first 24-48h
Defibrillation
Reperfusion
-blockers
Amiodarone 24-48h
Reperfusion
-blockers
VF or SMVT badly
tolerated beyond 48h
Defibrillation/cardioversion
Revascularization if indicated
ICD implant
Treatment of heart failure
Amiodarone or ablation in special
cases
SMVT well tolerated

with normal or quasi
normal LVEF
Cardioversion
Anti-arrhythmics
(procainamide or amiodarone)
-blockers
VT ablation
Consider ICD
Amiodarone in special cases
Reperfusion
-blockers
Symptomatic NSVT
-blockers
Amiodarone in refractory cases
-blockers
Amiodarone in special cases
Valuate the indication of ICD implant
as primary prevention
Reperfusion
-blockers
Asymptomatic
NSVT or ventricular
extrasystole
-blockers
Avoid anti-arrhythmics of Ic group
-blockers
Valuate the indication of ICD implant
as primary prevention
Reperfusion
-blockers
Torsades de Pointes
Defibrillation if sustained
Reperfusion
Magnesium
Shorten QT (pacemaker,
isoproterenol)
Correct electrolytes
Reperfusion
Correct electrolytes
AIVR
No (indicates reperfusion)
Atropine if symptomatic
No
No
Reperfusion
-blockers
ICD implant :
- LVEF < 40%, SMVT and
inducible NSVT
- CF II-III and LVEF < 35%
- CF I and LVEF < 30%
Table 37. Treatment of ventricular arrhythmias in STE-ACS

Atrial fibrillation occurs in 10% to 20% and is associated with major
infarctions with important ventricular dysfunction mainly in the elderly.
Anticoagulation is indicated due to the embolic risk involved.
Conduction disturbances
Almost 10% of STE-ACSs are found with AV block, and are more related
to mortality (due to the myocardial extension producing the block than
the block itself, since the block associated with the inferior infarction
has a good prognosis; Table 38).
AV BLOCKS POST AMI
SUPRA-HISIAN
It may be mild or turn into cardiogenic shock, and may increase mortality
in the short and long term. Symptoms, signs, diagnosis and treatment
are similar to other heart failure-related clinical situations. Killip and Forrester classifications are used to categorize the clinical situation.
The main causes of arterial hypotension in inferior wall STE-ACS are
shown in Figure 68.
INFRA-HISIAN
Heart rate
40-60 bpm (narrow)
< 40 bpm (wide)
AMI Location
Inferior
Anterior
Atropine response
Responds
No response
Prognosis
Good
Poor
Table 38. Atrial ventricular blocks in AMI

Almost 20% of infarctions develop transitory bundle branch block,
which persists in 5% of cases. New-onset bundle branch block, mainly
left-sided, is related to extensive infarctions with severe systolic dysfunction and a poor prognosis.
46
Acute Heart Failure because

of LV Systolic Disfunction
Inferior AMI
with hypotension
RV infarction
Liquids
Inotropes
Reperfusion
Cardiac rupture
Fluids
Pericardiocentesis
Surgery
Bradycardia
Atropin
Pump failure
Rare: diuretics
Inotropes
Contrapulsation
Reperfusion
Figure 68. Causes and treatment of inferior infarction with arterial hypotension
Card iology
Mechanical Complications
These are included in Table 39, and in Figures 69, 70, 71, 72 and 73.
COMPLICATIONS
TYPICAL LOCALIZATION AND

PREDISPOSING FACTORS
SYMPTOMS AND DIAGNOSIS
TREATMENT
Free wall rupture
Anterolateral
Old woman
Hypertension
First infarction
Reperfusion and -blocker reduce
the risk
Electromechanical dissociation
High vein pressure
Echocardiography
Cardiopulmonary resuscitation
Immediate surgery
Ventricular septal
rupture
Anterior septum (anterior infarction)

Similar to rupture
Mitral regurgitation
Inferoposterior (posterior papillary

muscle)
If rupture, shock and acute

pulmonary edema. Unilateral edema
sometimes.
Murmur in apex (absent or mild in
severe cases)
Giant V wave in PCW
Echocardiography
Reperfusion
In mild or moderate cases,
surgery is to be delayed
IABP
Diuretics
Vasodilators
Severe cases: immediate surgery
Ventricular aneurysm
Apical (anterior infarction)

First infarction
Reperfusion ACE inhibitors and ARAII
reduce the risk
No risk of rupture
Double apical impulse

Persistent ST elevation
Associated with heart failure,
embolism or arrhythmias
ECG
Complications treatment
Pseudo aneurysm
Anterolateral
Thrombus-contained rupture
Chest pain
ECG
Immediate surgery
Right ventricular
infarction
Inferoposterior
Hypotension
High vein pressure and absence
of rales
Increased ST in V4R
ECG
Shock and acute pulmonary edema

Parasternal murmur
Oximetry gap in RV
Echocardiography
Immediate surgery
IABP
Diuretics
Vasodilators
Reperfusion
Intravenous fluids
No diuretics, no vasodilators
Inotropes
Table 39. Mechanical complications of infarction
Figure 70. Repair of ventricular septal rupture because

Figure 69. Free wall rupture
of septum rupture in AMI
47
Others
Postinfarction ischemia requires immediate intervention and an early
coronarography.
Thrombosis (apart from those related to supraventricular arrhythmias)
is commonly related to the formation of intracavitary thrombus present
in almost 5% of infarctions, mainly anterior infarctions.
Meta infarction acute pericarditis (epistenocardiac) produces ST elevation
and pericardial pain, requiring NSAIDs or colchicine for the treatment.
Figure 71. Mitral regurgitation correction by papillary muscle rupture
Conventional
suture
Dressler syndrome is defined by fever, pneumonitis and polyserositis (pleuropericarditis) probably immune-mediated. It is equivalent to the post pericardiotomy syndrome appearing, in some occasions, after cardiac surgery.
It generally occurs between the first and second week after infarction,
though it may be delayed. Treatment is similar to that of pericarditis.
Recurrence is frequent (especially in patients who have been administered glucocorticoids, which are very ecient in relieving symptoms).
Colchicine may be useful to prevent recurrence.
Dyslipidemia
Suture
in inverted T
Chapter 08
Although a comprehensive study of dyslipidemia is included in the Endocrinology chapter, some considerations must be mentioned.
Pericardial
patch
LDL elevation is related to a greater risk of coronary heart disease, and it

is similarly related to VLDL. Levels of HDL are inversely related to development of premature arteriosclerosis. Factors leading to a rise in HDL levels
are physical exercise, lipid-lowering diet and female sexual hormones,
whereas factors lowering HDL levels are obesity, sedentary and smoking.
Figure 72. Repair of ventricular aneurysm (aneurysmectomy)
Saturated fatty acids (common in animal fat)

cause an increase LDL cholesterol, whereas
monounsaturated and polyunsaturated acids
cause an increase HDL. There are many drugs
to decrease LDL, such as statins. Others are
ezetimibe or resins, such as cholestyramine,
though these are hardly used nowadays due to
the adverse events produced.
There is a close relationship between abdominal
obesity (abdominal waist diameter over 102 cm
in men and over 88 in women) and CI; hypercholesterolemia, hypertriglyceridemia and hypertension are also associated.
Figure 73. Dierences between aneurysm and pseudoaneurysm
48
Metabolic syndrome is defined as a group of

cardiovascular risk factors appearing more frequently than usual. Metabolic syndrome components are hypertension, abdominal obesity, hypertriglyceridemia, lower HDL levels and altered
baseline glycemia.
Card iology
The metabolic syndrome pathophysiologic basis seems to be related to

insulin-resistance due to abdominal obesity. Nowadays, there is a debate
over whether metabolic syndrome adds another risk to the existing cardiovascular risks, although preliminary data suggests so, at least, in some
cases. Treatment includes adequate diet and regular physical exercise.
The latest guidelines for the treatment of blood colesterol to reduce atherosclerotic cardiovascular disease identify four groups of patients that
should be treated with statins:
Individuals with clinical atherosclerotic cardiovascular disease.
Individuals with LDL-cholesterol levels > 190 mg/dL, such as those
with familial hypercholesterolemia.
Individuals with diabetes aged 40-75 years old with LDL-cholesterol
levels between 70-189 mg/dL and without evidence of atherosclerotic cardiovascular disease.
Individuals without evidence of cardiovascular disease or diabetes
who have LDL-cholesterol levels between 70-189 mg/dL and a 10year risk of atherosclerotic cardiovascular disease > 7.5%.
Statins are classified according to the expected reduction of LDLcholesterol levels in high-, moderate- or low-intensity therapy (Table 41).
Actually, there is no evidence from randomized controlled trials to support treatment to a specific target of LDL- or HDL-cholesterol levels. So,
it is recommended to start and mantain statin treatment (drug and dose)
according to the previously reported groups:
Individuals with clinical atherosclerotic cardiovascular disease or those
with LDL-cholesterol levels > 190 mg/dL: high-intensity statin such be
used to attain at least a 50% reduction in LDL-cholesterol unless otherwise contraindicated or when statin-associated adverse events are present (in that case we should use a moderate-intensity statin therapy).
Patients with diabetes aged 40-75 years old with LDL-cholesterol
levels between 70-189 mg/dL should be treated with a moderateintensity statin (lowers LDL-cholesterol 30% to 49% from baseline).
Individuals without evidence of cardiovascular disease or diabetes
who have LDL-cholesterol levels between 70-189 mg/dL and a 10year risk of atherosclerotic cardiovascular disease > 7.5% should be
treated with a high- or moderate-intensity statin therapy.
The use of other lipid-lowering agents such as niacin or ezetimib has not been
shown to reduce the risk of atherosclerotic cardiovascular disease. (Table 40).
CALORIES RELEASED
ACTIVE
CONTRIBUTION TO DIET (%)
IN COMPLETE
SUBSTANCE
OVER TOTAL CALORIES
OXIDATION
Carbohydrates 4.1 kcal/g
50% to 65%%
Fat
9.3 kcal/g
Saturated: < 10%
Polyunsaturated: < 10%
Monounsaturated: 10% to 15%
Cholesterol
< 300 mg/day
Proteins
4.3 kcal/g
10% to 20%
Physical exercise prevents elevation of cholesterolemia and keeps HDL

levels high. In order to carry out a selective detection of hyperlipidemia, a
cholesterol blood test is recommended for all adults, especially for young
individuals with a family history of premature ischemic cardiopathology.
Cholesterol determination is recommended at least in men under 35
years of age and in women under 45 years of age. From that age on, an
analytic determination every five or six years is recommended.
HIGH-INTENSITY
STATIN THERAPY
Daily dose lowers
LDL-cholesterol
on average,
by approximately
> 50%
Atorvastatin (40)80 mg
Rosuvastatin 20(40) mg
MODERATE-INTENSITY
STATIN THERAPY
Daily dose lowers LDLcholesterol on average,
by approximately
30% to 49%
LOW-INTENSITY
STATIN THERAPY
Daily dose lowers
LDL-cholesterol
on average,
by < 30%
Simvastatin 10 mg
Pravastatin 1020 mg
Lovastatin 20 mg
Fluvastatin 2040 mg
Pitavastatin 1 mg
Atorvastatin 10-(20) mg
Rosuvastatin (5)-10 mg
Simvastatin 20-40 mg
Pravastatin 40-(80) mg
Lovastatin 40 mg
Fluvastatin 40 mg bid
Pitavastatin 2-4 mg
Table 41. High, moderate and Low-Statin Therapy. ( ) indicates less

evidence
Hypertension
Chapter 09
9.1. Essential Hypertension

High blood pressure or hypertension is highly prevalent (30% to 45%
in the general population) and has a steady incidence. Almost half the
people with hypertension are unaware of the condition and, in spite of
new therapeutic advances, optimum control is achieved in less than half
of patients; it is therefore a first-order public health issue.
Elevations in systolic and diastolic blood pressure are related to this cardiovascular disease systolic blood pressure (SBP) being a stronger sign
of potential complications than diastolic blood pressure (DBP) after 50
years of age-, and pulse pressure (dierence between systolic and diastolic pressure) in elderly patients also adds predictive value.
Table 40. Active substances proportion in normal diet
The most frequent form of HBP is essential (usually starting between

30 and 50 years of age, although starting to appear in younger people,
even teenagers). Among younger people, secondary causes such as aortic coarctation should be ruled out, and among elderly patients, it is convenient to rule out other etiologies such as renal artery atherosclerosis.
Hypercholesterolemia reduction is followed by a reduction in arteriosclerosis progression. The initial treatment of hyperlipidemia is a lowfat diet (< 30% to 35% of total caloric intake). Likewise, cholesterol in
saturated fatty acids and polyunsaturated acids must be poor. It is necessary that most fat be ingested by way of monounsaturated fatty acids
since they increase HDL levels (Table 41).
By consensus, adult high blood pressure (HBP) is defined as the presence of SBP over 140 mmHg (systolic HTN) and/or DBP over 90 mmHg
(diastolic HTN). However, these limits are arbitrary since there is a connection with cardiovascular risks at lower values, especially in patients
with high cardiovascular risk. Complete patient risk level should be determined, not only the BP level, to apply the correct therapeutic action
49
three drugs (including a diuretic). It may comprise up

to 15% of patients and the
main causes are inadequate
compliance with nonpharmacologic instructions, use
of hypertensive substances
(liquorice, NSAIDs, corticosteroids, cocaine, etc.), sleep apTable 42. Classication of BP values
nea, unsuspected secondary
cause, irreversible injury to
(this is carried out using calculation tables of cardiovascular risk, such as target organs or volume overload (sodium ingestion, insucient doses
of diuretic, progressive kidney failure or hyperaldosteronism). In these
Framinghams risk score or Euroscore).
cases it is convenient to rule out white coat hypertension, pseudo
hypertension or the use of a cu of inadequate size.
The most common classification of BP values is shown in Table 42.
TYPE
Optimum
Normal
Normal-high
HTN level 1
HTN level 2
HTN level 3
Systolic isolated HTN
< 120
120-129
130-139
140-159
160-179
180
140
SBP (mmHg)
and
and/or
and/or
and/or
and/or
and/or
and
In pediatrics, there is hypertension with BP values over 95% percent,

adjusted to age and sex; values ranging between 90% and 95% are considered prehypertension.
Systolic isolated HTN, common among the elderly, is characterized
by values over 140 mmHg of systolic and below 90 mmHg of diastolic
pressure and is also correlated with morbidity and mortality. However,
systolic isolated HTN among young people has not been revealed to improve prognosis with treatment, data also suggests that this entity does
not progress to systolic/diastolic HTN in every case.
Malignant HTN is a hypertensive emergency with ischemic organic damage
(retina, kidney, heart or brain), especially papilledema on ophtalmoscopy.
Although the most frequent cause is untreated essential hypertension, secondary causes of HTN must be thoroughly analyzed. Prognosis without treatment is 50% mortality in the first year. For treatment, intravenous drugs such
as labetalol, nitrates, nitroprusside or nicardipine are used.
Accelerated hypertension refers to patients who do not have papilledema but who have other severe vascular damage signs, such as hemorrhages or retinal exudates.
Hypertensive crisis are SBP elevations > 180 mmHg and/or DBP > 120 mmHg.
There are two types: hypertensive emergency and hypertensive urgency.
Hypertensive emergency describes an elevation in blood pressure accompanied by severe acute damage to target organs threatening the patients
life (encephalopathy, acute coronary syndrome, acute heart failure, aortic dissection, ischemic or hemorrhagic stroke, pheochromocytoma crisis,
drugs such as cocaine, amphetamines or MDMA, eclampsia or perioperative hypertension). The magnitude and speed for correction of pressure
values will depend on the involvement of the organ at issue. For instance,
for a stroke, correction will be small and slow, whereas in a case of acute
pulmonary edema or aortic dissection, the correction must be major and
quick. However, in most cases it is sucient with a 25% correction of the
BP values during the first hours and slowly thereafter, since a sudden and
intense BP drop could cause greater organic and ischemic damage. The i.v.
drugs used are similar to the ones used for malignant HTN. When a major
BP elevation is not accompanied by severe, acute organic damage, there is
hypertensive emergency. It is usually managed by restoring or increasing
antihypertensive therapy (in many cases it is the result of a break in use).
Resistant or refractory hypertension occurs when values cannot be
lowered to desired levels in spite of lifestyle changes and after at least
50
DBP (mmHg)
< 80
80-84
85-89
90-99
100-109
110
90
White coat HTN or isolated clinical hypertension defines patients

with normal BP values outside of medical consultation -determined
by ambulatory blood pressure measurement (ABPM), see below- and,
however, with consistently high values during consultation. It aects
15% of the population. It involves lower risk of damage to target organs
than the real hypertension, but apparently greater than people with
normal BP, therefore requiring close follow up and even consideration
of application of treatment if vascular risk is high or there are signs of
damage to target organs.
Masked HTN or isolated ambulatory HTN is the contrary phenomenon
(normal values during consultation, but higher elsewhere). It is just
as prevalent as clinical isolated HTN and is frequently associated with
damage to some target organ, therefore requiring similar management
as HTN.
Exertion HTN is characterized by normal BP at rest, but with excessive
increase during exertion (there is no consensus, but most define it as
SBP > 210 mmHg among men and > 190 mmHg among women). Its
usefulness for diagnosis and prognosis of patients with HTN is controversial. In the event of abnormal results, ABPM is advisable.
Measurement of Blood Pressure

A correct measurement of blood pressure is based on the auscultatory
method of Korotko sounds. Pseudo hypertension appearing with atherosclerosis may be suspected by palpating the radial artery with the
inflated cu (Oslers sign).
Diagnosis of HTN requires several elevated values over several days, except in extreme cases.
Measurement of ambulatory blood pressure (ABPM) -with automatic
devices- which takes multiple measurements throughout the day, appears to have greater correlation with some organic damage markers
and morbimortality. ABPM can be useful with certain patients, for instance, to assess the degree of control over BP values during valley
periods of drug eect, for the diagnosis of white coat HTN or masked
HTN, or for detecting nondipper patients whose BP during sleep does
not fall and who appear to have higher risk. However, it should be noted
that mean BP values obtained with this method are lower than those
obtained during oce visits; therefore HTN diagnosis will not be defined with values over 140/90 mmHg, but over 130/80 mmHg, 135/85
mmHg during the day and 120/70 mmHg at night.
Card iology
Self-measurement of blood pressure at home (SMBP) with automatic

devices that are correctly calibrated is more closely related the organic
damage caused by HTN than with measurement at oce visits, as well
as with a better estimate of cardiovascular (CV) morbimortality (similar
to ABPMs). In addition, it is useful with certain patients, since it has the
benefit of avoiding the white coat eect and shows the variation in BP
values after, for instance, altering drug therapy. In this context, normal
BP values are lower than those determined at oce visits, thus values
over 135/85 mmHg are considered HTN.
Basic complementary studies to be performed on every hypertensive
patient include blood and urine analysis, and ECG (Table 43). Other
tests such as echocardiography, chest X-ray, ankle-brachial index, carotid ultrasound or ophthalmoscopy, are advisable for many patients, as
well as other specific studies to determine suspected secondary causes.
ADVISABLE FOR ALL PATIENTS

Blood analysis: fasting glycemia, cholesterol and its fractions,
fasting triglycerides, potassium, uric acid, creatinine, hemoglobin
and hematocrit. Assessment of creatinine clearance or glomerular
filtration rate using appropriate formulas
Urine analysis: microscopic analysis and microalbumin test (reagent
strip)
ECG: to assess the presence of arrhythmia and signs of left-sided
ventricular hypertrophy
ADVISABLE FOR MANY PATIENTS
Echocardiograph (size of ventricular hypertrophy)

Carotid ultrasound (intima media thickness)
Chest X-ray
Ankle-brachial index
Ophthalmoscopy
Quantitation of proteinuria if reagent strip was positive for
microalbumin
Glucose tolerance test if glycemia is over 100 mg/dL
ABPM and/or SMBP
Pulse wave velocity calculation
GRADE I
Arteriolar constriction and sclerosis
GRADE II
Arteriovenous nicking
GRADE III
Exudates and hemorrhages
GRADE IV
Papilledema
Table 44. Hypertensive retinopathy. Keith-Wagener-Barker

classication
Hypertensive heart disease usually shows ventricular hypertrophy and

predominantly diastolic failure, although in advanced stages of severe
hypertension it can cause dilation and systolic ventricular dysfunction,
mimicking a dilated cardiomyopathy.
Atrial fibrillation is the arrhythmia most closely related to hypertension,
due to retrograde elevation of left-sided pressures. Further, hypertension is a stroke risk factor in atrial fibrillation.
Treatment
Hypertensive patients must comply with lifestyle and dietary instructions (low salt content, avoid overweight, perform exercise,
avoid anti-inflammatories) and monitor other cardiovascular risk
factors.
To decide when to start drug therapy for hypertension, systolic and diastolic pressure values alone are not enough in every case. It is advisable to assess the presence of established clinical or subclinical damage
of target organs and determine the overall cardiovascular risk (which
includes analyzing the presence and state of other cardiovascular risk
factors).
The latest recommendations for hypertension treatment published
by the Joint National Committee of United States are included in the
table below; there are slight dierences with other available guidelines
(Table 45).
Table 43. Complementary examination of hypertensive patients

RECOMENDATIONS FOR HTN TREATMENT
The main target organs with damage caused by hypertension are the
central nervous system (typically strokes), heart (hypertrophy, myocardial ischemia or arrhythmia) see Figure 74. Also, kidneys (renal failure
because of nephroangiosclerosis) and the retina (hypertensive retinopathy), Table 44.
Figure 74. 12-lead ECG of hypertensive patient with left-sided ventricular

hypertrophy and secondary repolarization alterations
Drug therapy is advised for adults over 60 years of age if

their systolic blood pressure (SBP) > 150 mmHg or diastolic
(DBP) > 90 mmHg. If SBP values obtained are < 140 mmHg
and there are no adverse effects, treatment does not need
to be adjusted
In patients under 60 years of age, drug therapy is advised to reduce
DBP < 90 mmHg. With lower levels of scientific evidence, it is
advisable to start treatment to lower SBP < 140 mmHg
In patients with chronic nephropathy or diabetes over 18 years
of age, drug therapy aims for blood pressure < 140/90 mmHg
For the general population (including diabetic patients) treatment
may start with thiazides, calcium antagonists, ACE inhibitors
or angiotensin II. For African American patients (including diabetics),
thiazides or calcium antagonists are advisable to commence
therapy. In case of chronic nephropathy, ACE inhibitors or
angiotensin II are recommended to start therapy
If targets are not attained within a month, it is recommended to
increase doses of the drug of choice or add a new drug (assess this
to start with if BP > 160/100 mmHg). If targets are not met with
three drugs referring the patient to a unit specialized
in hypertension is recommended
Table 45. Recommendations for HTN treatment of the JNC-8 (2014)
51
The therapeutic objective of hypertension treatment is to lower morbimortality of the patient; for this purpose an operational goal is followed
aimed at lowering blood pressure to values below 140/90 mmHg in
general, being lower in diabetic patients, and with more leniency with
regard to SBP in elderly patients.
The benefit of treatment is more closely related to lowering BP itself
than with a specific drug. For this reason, it is considered that all antihypertensive drugs are first line and all combinations are accepted,
except for ACE inhibitors with angiotensin II. However, the only drugs,
compared against placebos, which have been statistically shown to decrease morbimortality, are diuretics and beta blockers. Newer drugs
have not replicated this since it is unethical to design new studies
against placebo.
For patients with mild blood pressure elevation and low-moderate cardiovascular risk, treatment usually starts with a single drug in monotherapy and doses are subsequently adjusted or other drugs are combined to keep blood pressure values under control. However, patients
with markedly elevated blood pressure and high-very high cardiovascular risk usually require starting with antihypertensive therapy directly,
with a combination of two drugs and later adjustment of doses or addition of a third drug.
Table 46 shows the main cases for or against the use of each drug
group.
DRUG
-blockers
Thiazides
ADVISABLE
Coronary disease
Tachyarrhythmia
Heart failure
Hyperthyroidism
Pregnancy (second line)
Heart failure
Elderly
African American
Asthma
Bradyarrhythmia
Severe claudication
Diabetes risk
Metabolic
syndrome
Gout
Diabetes risk
Metabolic
syndrome
Pregnancy
Renal artery
bilateral stenosis
Hyperkalemia
Calcium
antagonists
Dihydropyridine
in monotherapy
for myocardial
ischemia
Constipation
-blockers
Benign prostatic
hypertrophy
Pheochromocytoma
Heart failure
Orthostatic
hypotension
Table 46. Situations that encourage or discourage the use of various

drugs for hypertension
52
Beta blockers are preferred in coronary patients, with atrial fibrillation or other tachyarrhythmias, hyperthyroidism, heart failure,
sometimes migraine or if there is essential tremor. They are not the
best option in case of high risk of developing diabetes (metabolic syndrome).
Calcium antagonists are preferred in cases of isolated systolic HTN
in the elderly, African Americans, angina with contraindication for
beta blockers, atrial fibrillation or, sometimes, for patients with migraine.
In resistant HTN cases, the application of potassium-sparing diuretics
such as spironolactone/eplerenone and amiloride or alpha blockers
such as doxazosin should be considered. In case medical treatment fails,
invasive procedures, such as renal denervation and carotid baroreceptor stimulation may be considered.
9.2. Secondary Hypertension
NOT ADVISABLE
ACE inhibitors
Heart failure
or angiotensin II Systolic dysfunction
Diabetes
Proteinuria
Chronic kidney failure
Renovascular HTN
Metabolic syndrome
Ventricular hypertrophy
Angina
Elderly
African american
Intermittent claudication
Metabolic syndrome
Ventricular hypertrophy
Pregnancy (second line)
ACE inhibitors are the drugs of choice in the presence of diabetes mellitus,
renovascular hypertension, and diabetic nephropathy -or from another
source-, ventricular systolic dysfunction or severe heart valve failure,
myocardial infarction history or high risk of developing diabetes. In case
of cough or angioedema caused by ACE inhibitors, the alternative is angiotensin II, which is useful when there is major ventricular hypertrophy.
The most frequent cause of secondary hypertension is renal (renovascular or renal parenchymaytous).
Kidneys are both involved as victims (they develop nephroangiosclerosis)
and executioners (pathogenesis involvement) in essential hypertension.
Many drugs (vasoconstrictors, hydrosaline retention facilitators such as
anti-inflammatories, and so forth) and endocrine and metabolic disorders (hyperthyroidism, Cushings syndrome, among others) cause hypertension.
Figure 75 lists the main causes of secondary HTN.
When HTN is secondary to an identifiable etiology, a treatment should
be conducted, if possible, according to the cause of the disease. For
instance, in case of renovascular hypertension, an angioplasty of the artery involved, or its surgical repair may be prescribed. However, medial
fibromuscular dysplasia (a nonatherosclerotic, noninflammatory vascular disease that causes abnormal growth within the wall of the renal
artery), HTN tends to remain stable and does not require mechanical
treatment if blood pressure is controlled by means of drug treatment.
When subadventitial fibrodysplasia is involved, this tends to be progressive and more frequently requires angioplasty or surgery.
9.3. Special Cases

In African American patients, diuretics and calcium antagonists appear
to oer more protection than other drug groups.
Card iology
Neurogenic
alterations
Drugs
Hyperthyroidism
Aortic coarctation
Atherosclerosis
Arteriovenous
fistules
Aortic regurgitation
Suprarenal
alterations
Pregnancy
Kidney alterations
to baseline levels in the third trimester. For HTN diagnosis during

pregnancy, the limit is defined for values over 140/90 mmHg, although ABPM may be more accurate. Hygiene and dietary steps are
recommended, including strict supervision and limitation of physical exercise (sodium restriction, weight loss or dietary supplements
are not advised during pregnancy). In the presence of gestational
hypertension with or without proteinuria, the use of drugs is recommended for values over 140/90 mmHg. Drugs of choice are methyldopa, labetalol or, less frequently, calcium antagonists (they may
delay birth) or beta blockers (especially metoprolol, with low risk of
intrauterine growth retardation). ACE inhibitors and angiotensin II
are contraindicated due to their potential teratogenic eects. Values over 170/110 mmHg require emergency admission, with the
use of intravenous nitroprusside or labetalol, oral methyldope or
nifedipine. Hydralazine use in this situation has been discontinued
because of higher perinatal risks than with other drugs. In case of
pulmonary edema, nitroglycerine is the drug of choice.
Pericardial Disease
Chapter 10
Figure 75. Main causes of secondary hypertension
For isolated systolic hypertension in advanced age, the drugs of choice
are calcium antagonists or thiazides. Nevertheless, other first-line
drugs may be used, avoiding abrupt BP falls and monitoring orthostatic hypotension with more lenient goals than with other hypertensive
patients.
In left-sided ventricular hypertrophy and in metabolic syndrome, the
drugs of choice are ACE inhibitors, ARB or calcium antagonists.
Alpha blockers (alpha-adrenergic-antagonists) are advisable for pheochromocytoma.
For benign prostatic hypertrophy, alpha blockers are advisable (although they may be less safe than other antihypertensives in monotherapy).
Patients with previous stoke may, in general, use any drugs. However,
treatment should be avoided during the first week after a stroke, unless
there is very high blood pressure.
Hospital admission is not required for hypertensive emergencies; initial treatment is applied with oral drugs such as captopril, furosemide
or amlodipine. Fast acting nifedipine should not be considered, since it
may cause hypotension and trigger myocardial ischemia.
Hypertensive emergencies require immediate treatment with intravenous drugs in an intensive care unit, being careful not to cause an
abrupt decline in BP to prevent hypoperfusion of vital organs. Nitroprusside, nitroglycerine, furosemide, urapidil and labetalol are highly
suitable drugs in this context.
With regard to pregnancy, it should be noted that during the second trimester BP values drop approximately 10-15 mmHg, returning
10.1. Acute Pericarditis

Pericarditis is inflammation of the pericardium. It is called acute pericarditis when signs and/or symptoms of pericardial inflammation exist for
one or two weeks (less than six weeks).
More than 80% of cases are idiopathic and a viral etiology is supposed
in most cases. However, there are many disorders that could be associated with pericarditis (Table 47).
The main symptom is retrosternal pain originating in the parietal pericardium with pleuritic characteristics. Pain severity increases by deep
inspiration, coughing and exercise, and decreases when the patient
leans forward. It is important to distinguish retrosternal pain from the
pain generated by other conditions, especially by acute myocardial infarction (Table 48).
Radiation of the pain towards one or both trapezius ridges is characteristic of pericarditis, but it is very rare in cases of acute myocardial
infarction. However, the pain may be absent when the etiology is tuberculosis, neoplastic, radiation or uremia.
A pericardial friction rub is very specific, resembling the sound of
squeaky leather, often reported as grating, scratching, or rasping.
It is usually best heard at the left sternal border when the patient
leans forward and during expiration, preferably in systole (sometimes it can also be heard during early diastolic filling or during
atrial contraction). It is not constant, it may be intermittent according to chest position or other factors. There may be fever often appearing simultaneously with the pain. Tachycardia and malaise are
common.
53
CAUSES
Idiopathic (over 80%)
Infectious
Virus: Coxsackie A and B, echovirus, adenovirus, infectious mononucleosis, chickenpox,
B hepatitis and HIV infection
Bacterial: tuberculosis, pneumococcus, Hemophilus, staphylococcus, streptococcus, gramnegative, Borrella and mycobacteria
Fungi: Histoplasma, Coccidioides and Candida
Others: amebiasis and toxoplasmosis
Transmural acute myocardial infarction
Chronic kidney disease
Malignancy
Metastatic neoplasms: lung cancer, breast cancer, lymphomas, leukemia and melanoma
Primary neoplasms: mesothelioma and lipoma
Post-radiotherapy syndrome
Autoimmune diseases: acute rheumatic fever, systemic lupus erythematosus, rheumatoid
arthritis, scleroderma, polyarteritis nodosa, Wegeners disease, Dresslers syndrome
and postpericardiotomy pericarditis
Inflammatory processes: sarcoidosis, amyloidosis and inflammatory bowel disease
Trauma: cardiac trauma, cardiopulmonary resuscitation, iatrogenic hemopericardium (implanted
pacemakers or defibrillators, angioplasty, endomyocardial biopsy, catheter ablation, etc.)
Drugs: hydralazine, procainamide, diphenylhydantoin, isoniazid, phenylbutazone, dantrolene,
doxorubicin, methysergide, penicillin, cromolyn and minoxidil among others
Other: myxedema, chylopericardium and aortic dissection
Table 47. Causes of pericardial disease

ACUTE PERICARDITIS
ACUTE MYOCARDIAL INFARCTION
ECG
The ECG is the most useful diagnostic test. It

can reveal:
Concave ST segment elevation, diuse in
almost all leads. As days go by, the ST segment returns to normal and the T waves
become negative (they may remain like
that for weeks or months). Clinical course
over time is very characteristic, although
the full sequence can be observed in less
than half of patients.
There may be a decrease in QRS complex
voltage and electrical alternans (variation
in the amplitude of the QRS complex) when
there is a large pericardial eusion associated.
There may be a PR segment depression, a
sign of atrial involvement. This sign shows
high specificity but low sensitivity for the
disease. Atrial arrhythmias can also be observed.
Chest x-ray and echocardiography may be normal, unless there is a severe pericardial eusion (Figure 76).
Speci ic forms
of pericarditis
Idiopathic pericarditis
ST
PR
Diffuse ST segment elevation (concave)

Sometimes depression
Localized ST segment elevation (usually

with reciprocal changes)
Rare elevation
Changes
T waves become inverted after the ST
in evolution segment becomes isoelectric
T waves can become inverted when

the ST segment is still elevated,
Q waves evolve
Pain
Pleuritic, sharp, stabbing, positional

changes, postural changes, radiating to
the back, to one or both trapezius ridges
Oppressive, often associated with

vegetative symptoms, radiates to the
left arm, jaw and other areas
Fever
It often appears on the days before or

simultaneously
If it appears, it is usually a few days after

the AMI
Table 48. Dierential diagnosis of acute pericarditis and AMI
Idiopathic pericarditis is the most common cause of acute pericarditis and is usually caused by certain viruses, although no
systematic tests for their detection are performed. There are usually signs and symptoms
of infection of the gastrointestinal or upper respiratory tract during the previous days or simultaneous to the disease. It is characteristic
of young adults and is sometimes associated
with pleuritis and/or pneumonitis. It does not
usually evolve into pericardial eusion with
cardiac tamponade or constrictive pericarditis.
For treatment, NSAID (ibuprofen, ASA in high
doses and indomethacin) are prescribed for
several weeks with a gradual dose reduction.
In rare cases, glucocorticoids or colchicine
are required to treat inflammation and opioid
analgesics for pain. Anticoagulants should be
avoided because of the risk of hemorrhagic
transformation. It usually heals without sequelae.
Figure 76. Echocardiography image of severe pericardial eusion (PE) in apical (A) and subcostal
(B) four-chamber view
54
The major problem in this condition is the tendency to relapse (25% of cases). Colchicine is
the preferred drug for preventing relapse, administered during the acute phase and several
weeks later, in association with a NSAID, or
Card iology
alone. When recurrences are frequent, pericardiectomy could be evaluated.
Dresslers Syndrome
and Postpericardiotomy
The former usually occurs the first few weeks after myocardial infarction and the second after cardiac surgery or trauma. Both seem to
have an autoimmune etiology and are characterized by fever, pericarditis, pleuritis, pneumonitis and, sometimes, arthralgia. Anticardiomyocyte antibodies are frequently detected. Treatment is similar
to idiopathic pericarditis. Recurrence is also very common, especially
if glucocorticoids have been administered. A transmural infarction or
cardiac trauma can also lead to pericarditis with no autoimmune etiology, such as epistenocardiac pericarditis, during the early days of
the condition.
Other Etiologies
Connective tissue diseases especially systemic lupus erythematosus and rheumatoid arthritis, are frequently associated with asymptomatic pericardial eusion, although they may manifest as acute
pericarditis.
Acute rheumatic fever associated with pericarditis is often a manifestation of severe pancarditis.
Pyogenic pericarditis may occur after cardiothoracic surgery, in immunocompromised patients, esophageal rupture into the pericardium, by extension of foci of pyogenic infection of mediastinal or pulmonary origin, or within sepsis (sometimes sepsis is associated with
aseptic inflammatory pericarditis). Since pyogenic pericarditis has a
high mortality rate, early treatment with intravenous antibiotics is
required.
Tuberculous pericarditis manifests with pain (which is not usually
as intense as in idiopathic pericarditis), pericardial rub and fever.
There may be other signs of systemic tuberculosis infection (anorexia, weight loss, among others). Sometimes there is asymptomatic
chronic pericardial eusion, subacute or chronic pericarditis prone
to constriction. In addition to the tuberculosis treatment, NSAIDs
and, sometimes, corticosteroids are administered to prevent obstruction.
Uremic pericarditis is very common, appearing in about one-third
of patients with advanced kidney failure, especially in those undergoing dialysis. It is usually subacute and is frequently painless, although pericardial rub is often present. Fluid may be fibrinous or
hematic. Treatment includes anti-inflammatory drugs and hemodialysis. If there is cardiac tamponade, pericardiocentesis should be
performed. When pericarditis is recurrent or persistent, pericardiectomy may be indicated.
CCP can potentially occur as a result of any pericardial disease, but it

rarely occurs after acute pericarditis. In most cases, the origin is unknown, but among the most common known causes are cardiac surgery, mediastinal radiotherapy, tuberculous pericarditis, connective
tissue diseases, and recurrent uremic pericarditis.
Systemic congestion signs such as increased jugular venous pressure,
hepatosplenomegaly and edema are predominant. Hepatosplenomegaly is often accompanied by ascites and liver dysfunction, so it can
easily be confused with cirrhosis. Dyspnea is uncommon, although
there may be weakness and signs of malnutrition (systemic congestion may produce protein-losing enteropathy). Other signs that can
be detected include decreased apical impulse and intensity of heart
sounds. Sometimes a sound appears immediately after the second
sound, which corresponds to a pericardial knock and should not be
confused with a third sound. The Kussmaul sign (raised jugular vein
pressure on inspiration) is very common. A rapid y descent (prominent diastolic collapse of JVP) is usually noted followed by a rapid rise
and subsequent plateau, with a greater a wave and, sometimes, a
prominent x descent.
The ECG may reveal decreased QRS complex voltage, flattening and
inversion of T waves, and atrial fibrillation in about one-third of patients.
In about 50% of the patients, chest x-ray shows calcification of the pericardium, although this does not always causes constriction. An echocardiography generally shows thickening of the pericardium but, in case it
is normal, this diagnosis should not be ruled out.
Cardiac MRI and CT scans are more reliable for detection of pericardial thickening than echocardiography. However, pericardial thickening
does not necessarily indicate constriction (Figure 77).
Chronic Constrictive Pericarditis

Chronic constrictive pericarditis (CCP) is caused by chronic inflammation
of the pericardium. Granulation tissue, fibrosis and calcification may develop (the fibrous process may extend to the adjacent myocardium),
with subsequent loss of normal elasticity of the pericardial tissue and
impairing diastolic function. In the early phase of diastole, ventricular
filling is normal, but it is abruptly disrupted when the maximum elasticity of the pericardium is attained. Stroke volume decreases while there
is equalization of diastolic pressure in all four heart chambers.
Figure 77. CT scan from a patient with constrictive pericarditis showing

intense pericardial calcication
Catheterization is usually unnecessary although, in some cases, it

is performed to distinguish it from restrictive cardiomyopathy. The
x and y descents are prominent and a wave is usually increased, re-
55
sulting in an M shape. In jugular vein pressure, y descent is the

most prominent wave, interrupted by a rapid rise in pressure (called
h wave).
In the ventricle, diastolic pressure acquires a square root or dip-plateau
shape, since pressure increases rapidly when the limit of pericardium
distensibility is reached and it remains on a plateau.
This pressure pattern is very similar to the one in restrictive cardiomyopathy and, on rare occasions, endomyocardial biopsy may be required to dierentiate them. Left filling pressure is higher than right
filling pressure by more than 5 mmHg in restrictive cardiomyopathy (in
constrictive pericarditis they are usually almost equal); this dierence
is magnified by volume overload or Valsalva maneuver in restrictive
cardiomyopathy. BNP elevation may support the diagnosis of restrictive cardiomyopathy.
Pericardiectomy (pericardial stripping) is the only definitive treatment
for constrictive pericarditis. This procedure consists of performing a
longitudinal incision on the pericardium, finding a dissection plane
and removing it carefully to prevent myocardial tears or lesions of
the coronary vessels. If there is serious calcareous infiltration of the
epicardium, it is better to leave some portions than run the risk of a
complete removal.
The success of the intervention depends on the clinical course of the
condition since, in very advanced cases, pericardial stripping is very
complex and the improvement obtained is limited. Therefore, surgery
should be carried out at a relatively early stage.
Operative mortality is about 5% to 10%. Benefits obtained from complete
cardiac decortication are progressive, it is only after several months that
complete improvement can be observed and that jugular venous pressure returns to normal. Long term benefits are often very satisfactory.
Medical therapy consists of sodium restriction, diuretics, digoxin and, in
some cases, repeated pericardiocentesis. Due to the fact a large number of
cases of constrictive pericarditis are of tuberculous origin, this must be treated before surgery if suspected or this etiology cannot be ruled out. In case of
atrial fibrillation, anti-coagulation is indicated.
10.2. Cardiac Tamponade

This is caused by accumulation of fluid in the pericardial cavity that
compromises the filling of the ventricles by extrinsic compression.
The presence of fluid in the pericardium gradually increases the
pressure, thus becoming the main pathophysiologic factor (Figure
78).
The increase in pericardial pressure compresses cardiac chambers (first
the right atrium and the right ventricle during diastole, the moment of
lowest pressure) and produces collapse. This complicates the filling of
the right cavities and results in an increase in the pressure of the systemic venous territory (this is why in chronic pericardial eusion there
is systemic congestion) and a fall in cardiac output that may cause hypotension and signs of antegrade low output.
56
Figure 78. Pathophysiology of cardiac tamponade

The increase in pericardial pressure compresses cardiac chambers (first
the right atrium and the right ventricle during diastole, the moment of
lowest pressure) and produces collapse. This complicates the filling of
the right cavities and results in an increase in the pressure of the systemic venous territory (this is why in chronic pericardial eusion there
is systemic congestion) and a fall in cardiac output that may cause hypotension and signs of low anterograde cardiac output.
Since all chambers are surrounded by the pericardial sac, the increase
in volume results in higher pressure compensated by interdependence
of the chambers (Figure 79).
During inspiration, the negative pressure in the chest allows more blood
to return to the right chambers. In order to hold a greater volume in compressed cavities, there is distention of the right ventricle at the expense of
compression of the left ventricle by the septum, which can lead to paradoxic pulse. As a result of the tamponade, the whole diastole process is compromised (unlike cases of constrictive pericarditis, in which the first stage is
not aected).
The body adapts by activating compensation mechanisms analogous to
those of heart failure: activation of the sympathetic and renin-angiotensin-aldosterone system aims at increasing cardiac output and maintain
peripheral perfusion. In cases of acute tamponade (aortic dissection, cardiac rupture, etc.), intrapericardial pressure rises so fast that the possibilities of compensation are limited. In case of slow onset eusion, temporary compensation is possible, but additional minor pressure elevations
can trigger florid tamponade. The factors that determine the presence
or absence of signs of pericardial tamponade are directly related to the
magnitude and speed of the onset of the eusion and the stiness of the
parietal pericardium, and inversely related to myocardial thickness.
Any pericarditis is a potential cause of cardiac tamponade. The most
frequent are tumors, idiopathic pericarditis (it rarely produces tamponade, but since it is the most common cause of pericarditis, it has a high
Card iology
incidence in global tamponades), and pericarditis with uremic and iatrogenic etiology (in interventional procedures or cardiac surgery).
plex can be observed, as well as electrical alternans of the waves if

the eusion is considerable.
Chest x-ray. The cardiac silhouette may be normal or, if the eusion
is large, it may be increased in size or acquire a water bottle shape
most frequently, lung fields are clear, with minimal or no alveolar
infiltrate (Figure 80).
Normal heart
(the pericardium facilitates
ventricular distention)
Decreased pulmonary vascularization
Arterial pulse
Expiration - Inspiration - Expiration
Cardiac tamponade
(pericardial fluid prevents
ventricular relaxation; it exerts
pressure on the septum)
Enlarged cardiac silhouette with "tent shape"

Over
10 mmHg
Arterial pulse
Expiration - Inspiration - Expiration
Figure 79. Interdependence of the chambers in pericardial tamponade

Clinical manifestations are related to the decrease in cardiac output and
systemic congestion: hypotension, tachycardia and oliguria, increased
central venous pressure, dyspnea with orthopnea, liver congestion,
among other conditions Physical examination reveals:
Increased jugular venous pressure with a prominent X descent and
a reduced or almost absent Y descent. Dullness on percussion of the
anterior chest.
There is often paradoxic pulse (inspiratory decline in systolic blood
pressure of more than 10 mmHg). This sign is not pathognomonic
and can be observed in constrictive pericarditis (one-third of cases),
restrictive cardiomyopathy, hypovolemic
shock, asthma or severe lung disease, and
A
in other conditions of right ventricular diaPE
stolic involvement.
Kussmauls sign is characteristic of constrictive pericarditis, but it can also be
LV
RV
observed during cardiac tamponade, especially if there is a component of pericardial
constriction.
RA
LA
D
Pericardial rub is rare and, if present, it suggests the possibility of constrictive pericarditis.
Figure 80. X-ray of a pericardial eusion

Echocardiography. Diastolic collapse of the right cavities and striking changes in ventricular filling flow with respiration are echocardiographic signs of tamponade (Figure 81).
Also, during inspiration, there may be an increase in right ventricular diameter, a decrease of the left ventricular diameter and a smaller opening of the mitral valve.
Catheterization. This is not usually necessary for diagnosis, but it
shows increased right atrium pressure with a prominent X wave and a
smaller Y wave. Intrapericardial pressure is similar to right atrium pressure. Equivalent pressures: right atrium pressure, right ventricle
pressure, pulmonary artery pressure and pulmonary wedge pressure are all very similar.
B
PE
Expiration
Inspiration
Expiration
Inspiration
Complementary Studies
ECG. There is often reactive sinus tachycardia. Decreased amplitude of the QRS com-
Figure 81. Echocardiographic image (apical four-chamber view) showing severe pericardial
eusion (PE) in a case of cardiac tamponade (A). Doppler image of transmitral ow showing ample
respiratory variations in diastolic lling ow velocity (B)
57
Treatment
Expanding blood volume with serum or additional blood reduces collapse
of the cavities. Diuretics are contraindicated, since they reduce blood flow
volume and may increase collapse, which can induce shock. Treatment consists of evacuating pericardial fluid and, as a result, intrapericardial pressure
is reduced. In extreme cases, pericardiocentesis should be performed as
soon as possible. Drainage can be performed using two techniques:
Pericardiocentesis. Under local anesthesia, a long needle is inserted
between the xiphoid process and the left costal arch, guiding it toward the left shoulder. The puncture can be guided by ECG, echocardiography or fluoroscopy (Figure 82).
Increased life expectancy and reduction of rheumatic fever incidence in

industrialized countries have altered the prevalence and the predominant etiology of valvular heart disease (VHD); calcific aortic stenosis is
the most common, nowadays. An important detail regarding the prevalence and incidence is that studies usually refer to severe VHD requiring medical care, treatment or surgical intervention since mild VHD
constitutes an incidental finding in healthy individuals. For instance, up
to one-third of the population presents mild or low mitral or tricuspid
regurgitation with no risk of progressive deterioration. Likewise, rigid
valves, slightly degenerated or sclerotic, are commonly detected in old
patients, with mild or no functional alteration, whose progression risk
is hard to estimate.
According to the speed of onset, VHD may produce dierent clinical
events because of the dierent heart chambers, pulmonary vascularization and compensation mechanisms. For left VHD:
Acute onset (acute myocardial infarction, endocarditis, aortic dissection, prosthetic valve thrombosis) is generally poorly tolerated,
leading to heart failure with low cardiac output and pulmonary
edema. Therapeutic action must be immediate (generally surgery).
Progressive or chronic onset activates compensatory mechanisms,
with little or no symptoms, even maintaining normal ventricular
function until advanced stages.
Figure 82. Pericardiocentesis guided by uoroscopy in patient with
Primary VHD (consequence of rheumatic fever, degenerative diseases)

frequently progress, and surgical intervention becomes necessary sooner or later. Functional (secondary) VHD (related to dilation or to valve
system failure because of another reason) may reduce after treating the
primary cause.
cardiac tamponade
A pericardial catheter can be placed and left for several hours or days,
enabling continuous drainage. In some cases, it may also be helpful for
intrapericardial treatment. There is risk of puncture of cardiac cavities,
pleura or other structures.
Pericardial window. This technique is indicated for patients who need
something more than a simple puncture. When there are frequent
relapses, septation, and purulent eusion or in cases of uremic etiology, this is the preferred procedure. It is performed using a subxiphoid
approach or left thoracotomy. This surgical drainage has the advantage that it is more complete and a biopsy of the pericardium can be
performed. Since it is performed under direct vision, it is easier to
remove adhesions and localized eusions. Risk is minimal and longterm outcome is better than that of performing a puncture.
Valvular heart
Chapter 11
disease
11.1. Basic Concepts

Cardiac valves may be aected by congenital heart disease or by acquired heart disease (Figure 83).
58
Semilunar valves stenosis (pulmonary and aortic valves) produces an

elevated afterload compensated with concentric hypertrophy, maintaining a good systolic function until advanced stages (hypertrophy
stimulates the onset of coronary diseases). As a general rule, symptoms
appear before ventricular systolic dysfunction, suggesting that surgery
is to be performed.
Valve regurgitation produces volume overload with eccentric hypertrophy associated with progressive ventricular dilation, initially compensatory, and then becoming degenerative. It is frequently prior to
symptoms, for which close monitoring is required for asymptomatic patients. Likewise, this volume overload increases ejection fraction, and
an apparently normal value may indicate incipient myocardial systolic
dysfunction.
In atrio-ventricular stenosis (mitral and tricuspid) symptoms are caused
by the increase in atrial pressure and retrograde congestion of the vein
system (pulmonary in mitral stenosis and systemic in tricuspid stenosis).
However, ventricles are not aected since they are protected against
such pressure or volume, so, ventricular diastole, rather than systole,
is compromised.
VHD symptoms may be anterograde (low cardiac output data such as
asthenia, tendency to tiredness) or retrograde. In left VHD, mitral or
aortic, high blood pressure flows back into the pulmonary veins (resulting in dyspnea and pulmonary edema). These vessels become more
resistant, which initially protects the lung from pulmonary edema,
but subsequently if the edema persists, it leads to irreversible organic
changes in the pulmonary arteriole with the pulmonary hypertension
and secondary right-side heart failure.
Card iology
Figure 83. Fibrous skeleton of the heart and mitral valve

VHD may manifest by the onset of complications (emboli, endocarditis, arrhythmias or sudden death). Sometimes, ergometry is useful to
determine hidden symptoms, especially in people with poor physical
activity level.
Upon clinical suspicion based on exploratory findings, the first-choice
diagnosis method for all VHD is the echocardiogram, and the VHD is
classified as mild, moderate or severe according to its hemodynamic
severity. Apart from studying the hemodynamic degree of the valve, it
is recommended to analyze its anatomy and subvalvular apparatus in
an attempt to establish the etiology and healing possibilities. A study on
the impact over myocardial function (volumes and ejection fraction),
on the pulmonary vessels (pulmonary hypertension data), on systemic
veins (size and flow of inferior vena cava), and on potentially altered
structures associated with VHD (e.g. aortic regurgitation associated
with ascending aortic dilation or coarctation) must also be performed.
Mild or moderate VHD does not itself significantly aect cardiac function and does not require surgical treatment, except for special cases.
However, severe left VHD frequently requires surgical intervention. Al-
though there are some peculiarities included in the relevant chapter,

surgery is indicated in two cases:
Where symptoms exist.
In asymptomatic cases, where significant impact over the cardiac
function exists. In mitral stenosis, impact data appear in the atrium
(atrial arrhythmias) and in the pulmonary artery (pulmonary hypertension), since the left ventricle only suers a chronic diastolic
deficit. Mitral regurgitation and aortic stenosis or regurgitation implies chronic pressure overload (aortic stenosis) or volume overload
(regurgitation) of the left ventricle, which makes it fail (ejection fraction decreases). Surgery is indicated.
Table 49 shows a summary including etiology, symptoms and treatment of left valve heart diseases.
As a general rule, a presurgical coronarography is indicated when surgery is scheduled with the purpose of reducing perioperative morbimortality and assessing the need for myocardial revascularization associated with the valve surgery, in patients with coronary disease or risk
of acquiring it (Table 50).
59
SUMMARY OF VHD
In MR and AR, if LVEF < 30%: transplantation
MS
Rheumatic fever (RF)
ETIOLOGY
MR
RF (MS + MR)
Mitral valve prolapse
MI isolated
AS
< 30 years: unicuspid
30-70 years: bicuspid
>70 years: degenerative
Dyspnea
Angina
Syncope
Dyspnea (more frequent)
Diuretics
Digoxin
Nonvasodilators
ACE inhibitors
Nonvasodilators
ACE inhibitors/Dihydropyridine
calcium antagonists
LVEF < 60% and/

or LVESD 40 mm
although asymptomatic
Repair attempt: if not
possible, prosthesis
LVEF< 50% although

asymptomatic
Children: surgery always
PROSTHESIS
Alternative: Ross procedure
Children: valvuloplasty
Upon high surgical risk,
consider TAVI
LVEF < 50% or LVESD > 50 mm

or LVEDD > 65 mm although
asymptomatic
PROSTHESIS
Alternative: Ross procedure
In some cases, repair
Surgery upon symptoms

If PH although
asymptomatic
SURGICAL TREATMENT
Dilation of annulus:
Acute: aorta dissection
Chronic: Marfan syndrome
Dyspnea
Dyspnea
SYMPTOMS
MEDICAL TREATMENT
AR
Valvular:
Acute: endocarditis
Chronic: RF
Favorable anatomy:
PMBC
Unfavorable anatomy:
prosthesis
Table 49. VHD summary

PRESURGICAL CORONAROGRAPHY. INDICATIONS
Known coronary disease

Suspected unstudied myocardial ischemia
LV systolic dysfunction
Existence of any coronary risk factor
Men over 40 years of age or postmenopausal women
Suspicion of ischemic severe mitral regurgitation
The deterioration risk is higher in cases of renal insuciency, hyperparathyroidism, people under 60-65 years of age, or hypercholesterolemia, Figure 85.
Table 50. Indications of presurgical coronarography in VHD

Some concepts about valve prosthesis should be known:
Some prosthesis are made of biological material and others are purely
mechanical, Figure 84.
Figure 85. Deteriorated biological prosthesis

Mechanical prosthesis requires chronic anticoagulation in order to attain an INR between 2.5 and 3 and low-dose aspirin, especially mitral
prosthesis or those in the right side.
Figure 84. Biological prosthesis (left)
60
and mechanical prosthesis (right)
Biological prosthesis only requires anticoagulation (INR 2.5) in the first

three months until endothelialization of the prosthetic material.
Biological prosthesis on the left side of the heart (mitral and aortic)
presents risk of deteriorating in the long run, thus requiring intervention. When in the right side of the heart, the risk is lower.
It is appropriate to know the dierences (shown in Table 51) between

mechanical and biological prosthesis well, although the choice of one
type of prosthesis over the other must be individualized.
Card iology
MECHANICAL PROSTHESIS
BIOLOGICAL PROSTHESIS
No contraindications to chronic
anticoagulation
Patients with coagulation
indications for another reason
or with high embolic risk
(atrial fibrillation, mechanical
prosthesis in another valve,
severe left systolic dysfunction,
emboli family background,
hypercoagulability conditions)
High risk of valvular
deterioration
(hyperparathyroidism)
< 60 years of age
Patients with high reintervention
risk (left ventricular dysfunction,
prior bypass surgery, multiple
prosthesis)
Difficulties in or
contraindications for correct
chronic anticoagulation (high
hemorrhagic risk, lifestyle
or occupation, compliance
problems)
Resurgery because of prosthetic
thrombosis with proven poor
anticoagulation control
> 70 years of age
Young woman with pregnancy
desire
Express desire of informed
patient
LA-LV Gradient
LA Pressure
Pulmonary
congestion
Pulmonary
hypertension:
- Active
- Passive
RV
hypertrophy
LV Filling
Dilation
of LA
Blood
accumulates
Cardiac output
Atrial
fibrilation
Emboli
Right HF
Table 51. Favorable factors to implementation of each type

of prosthesis
Figure 86. Mitral stenosis pathophysiology
The main complications of valve prosthesis are prosthetic endocarditis

(premature or late), thrombosis (requiring surgery or fibrinolysis), prosthetic dysfunction (requiring surgery when needed), emboli and hemolysis (destruction of red blood cells because of collision of the elements
of the prosthesis, not common in current models unless a prosthetic
dysfunction exists).
The main exploratory findings are summarized in Figure 87. Remember

that the greater the severity, the more accelerated the opening snap
and the more delayed the closing. In the same way, the murmur presystolic accentuation is lost upon atrial fibrillation.
Mechanical prosthesis causes a normal metallic click when opening and

closing.
Almost all prosthesis are slightly restrictive and produce a murmur similar to mild stenosis of the relevant valve.
11.2. Mitral Stenosis

Mitral stenosis is considered severe when it becomes smaller than
1.5 cm2 (and very severe if 1 cm2). This usually corresponds to a transmitral mean gradient of > 5 mmHg to 10 mm Hg at a normal heart rate.
The main cause of mitral stenosis is rheumatic fever, and mitral insufficiency is commonly associated (double mitral injury).
Figure 87. Mitral stenosis auscultation
The main symptoms are secondary to the retrograde increase in pulmonary pressure causing dyspnea, overload of the right side of the heart
(including right valves regurgitation as a result of dilation of the annulus
and even right heart failure.
Atrial fibrillation associated with mitral stenosis is very common; this

association represents an extreme embolic risk.
In Figure 88, general guidelines for mitral stenosis are shown. Normally,
this disease progresses slowly over period of 10-20 years before producing symptoms. However, when symptoms appear, they progress in a few
years, which constitute a significant increase in mortality if no measures
are performed. This is why symptoms with ordinary activity are the base
of surgical indication. Likewise, in asymptomatic patients, percutaneous
mitral balloon commissurotomy (PMBC) can be performed in cases of atrial fibrillation, PCWP > 25 mmHg with exercise or in very severe stenosis.
Upon examination, malar flushing is observed and murmur auscultated

in the apex with the chestpiece, in mid-diastole after the opening snap
with presystolic accentuation (as long as the patient is in sinus rhythm).
Medical treatment consists of bradycardia (upon an increased diastolic

time, a greater LV preload occurs), diuretics and antithrombotic treatment, especially in case of atrial fibrillation.
The pathophysiology is shown in Figure 86.
61
Area > 1.5 cm2
Area > 1.5 cm2
PCWP >25 mmHg with exercise
PMBC
Periodic monitoring
Atrial Fibrillation
or area <1 cm2
Favorable for PMBC
Favorable for PMBC
PMBC
Yes
NYHA class III-IV

with high surgical risk
No
MVR
Figure 88. General Intervention in mitral stenosis

PMBC (surgical commissurotomy is no longer used) can be performed
when the valvular anatomy is favorable and there are no contraindications (left atrial thrombus or moderate-to-severe MR). If unfavorable
(generally when one of these three criteria is met), replacement of the
prosthetic valve is indicated.
output (lack of blood flowing onwards since it flows back into the left
ventricle) are also included.
Chronic mitral regurgitation murmur is better auscultated in the apex
or armpit; it is systolic and generally larger when regurgitation becomes
more severe, Figure 89.
11.3. Mitral Regurgitation

Nowadays, mitral regurgitation is the second most common VHD after
aortic stenosis. There are three types: organic, ischemic and functional.
Organic mitral regurgitation is produced by diseases aecting the
valve and/or the subvalvular apparatus, such as mitral valve prolapse and rheumatic fever (the latter, frequently related to stenosis).
Ischemic mitral regurgitation appears as a consequence of dysfunction
or rupture of a papillary muscle, or because of distortion of ventricular
geometry upon an infarction altering spatial position of papillary muscles and subvalvular apparatus, complicating the closing of the valve.
Functional mitral regurgitation is caused as a consequence of the annular remodeling in any cardiomyopathy with significant ventricular
dilation.
Severe acute mitral regurgitation is usually due to infective endocarditis, dysfunction/rupture of a papillary muscle during infarction, rupture
of tendinous cord in mitral prolapse or traumatisms, including iatrogenic
mitral regurgitation.
From a pathophysiologic view, symptoms of retrograde congestion
(ejection of blood retrograde into the atria) and that of low anterograde
62
Mitral focus
radiates armpit
MT
AP
S3
MT
Hyperflux murmur
Figure 89. Auscultation in mitral regurgitation

Severe acute mitral regurgitation usually runs with acute pulmonary
edema, cardiogenic shock, and S3 is common. Murmur may be short
or inaudible.
Card iology
Suspected mitral regurgitation

Anamnesis
Physical
examination
Echocardiogram
Mild
or moderate
Follow-up
Define etiology
Primary MR
Symptoms
Surgery
(attempt of repair
if possible)
Secondary MR
Coronary artery disease treatment,

heart failure treatment, consider CRT
Asymptomatic
EF 30% to 60%
and LESD <40 mm
or new onset AF
or PASP >50 mmHg
EF 30% to 60%
or LVESD 40mm
Symptomatic severe
MR with persistent
NYHA class III-IV
symptoms
Asymptomatic
severe MR
or progressive MR
Periodic monitoring
Likelihood of succesful
repair > 95% and expected
mortality < 1%
MV repair
MV surgery
(MV repair preferred)
Periodic monitoring
Figure 90. General Intervention in mitral regurgitation

In Figure 90, general measures of mitral regurgitation are shown.
Medical treatment includes diuretics for congestion, digoxin (positive inotrope) and vasodilators (ACE inhibitors) reducing preload and
afterload (in order to reduce regurgitation and to increase anterograde
output).
Surgery is indicated when symptoms appear (dyspnea, generally) or
upon the slightest evidence of deterioration of ventricle systolic function (ejection fraction < 60% or LV telesystolic diameter > 40 mm) since
it is irreversible once it occurs.
Mitral valve repair is preferred over mitral valve replacement when possible (more feasible in mitral valve prolapse and lesser in rheumatic mitral regurgitation) (Figure 91).
Concomitant mitral valve surgery (preferred repair) is reasonable in patients with chronic primary moderate MR when undergoing cardiac surgery for other indications or for patients with chronic severe secondary
MR who are undergoing coronary coronary artery bypass graft or aortic
valve replacement.
Usual treatment of mitral regurgitation because of annulus dilation is
that of advanced heart failure.
Figure 91. Mitral valve repair
63
Mitral valve prolapse (Barlow syndrome) usually produces a midsystolic click followed by a mitral-regurgitation-produced murmur
(click-murmur syndrome). When preload decreases (Valsalva, bipedestation), the click comes earlier and murmur duration extends
(Figure 92).
S1
children, it is the unicuspid aortic valve which predisposes to aortic stenosis.
S2
C
S1
S2
C
S1
S2
C
Figure 94. ECG of bicuspid aortic valve with evidence of deterioration in

both walls (parasternal short-axis plane)
Figure 92. Variations of mitral valve prolapse murmur

In significant organic mitral regurgitation, mitral prolapse related to the murmur involves a deterioration risk, especially in men
(Figure 93).
Aortic stenosis is considered severe when the aortic valve area is < 1.0
cm2 (or indexed AVA 0.6 cm2/m2). If systolic function and cardiac output are normal, aortic stenosis is severe as long as the mean systolic
transvalvular gradient exceeds 40 mmHg.
Upon systolic dysfunction, the ventricle cannot open the valve completely. The aortic valve area may still be compatible with aortic stenosis. Dobutamine stress echo is used to determine the gradient growth
with poor increase of the valvular area in cases of true aortic stenosis.
Characteristic symptoms of severe aortic stenosis are triggered by effort (angina, syncope or dyspnea). Nowadays, the main predominating symptom is dyspnea. The risk of sudden death in aortic stenosis
increases exponentially when symptoms appear. The latter indicates
the need for aortic valve replacement.
Aortic stenosis murmur is auscultated in the aortic focus, radiating to
carotids and supraclavicular area (occasionally the apex: Gallavardin phenomenon). It is systolic and rhomboidal after aortic opening
(sometimes an opening click may be heard, mainly in bicuspid valves).
As the murmur lasts longer, its severity also increases (Figure 95).
Figure 93. Echocardiographic image in an apical four chamber

view showing a mitral valve prolapse (arrow)
11.4. Aortic Stenosis
Aortic valve sclerosis without stenosis is common in elderly patients

with hypertension and produces a low intensity and short murmur. Severe aortic stenosis is usually accompanied by a decrease in intensity
and inverted closure in S2. It is also common to indentify a parvus et
tardus pulse.
Figure 96 shows general measures for patients with aortic stenosis.
Degenerative aortic stenosis in the elderly is the most common valvular

heart disease in developed countries, frequently associated with atherosclerosis.
Bicuspid valve usually predisposes to aortic stenosis at middle-age
(Figure 94), as a result of early degeneration over the years. In
64
Medical treatment is of limited use in severe aortic stenosis. Surgery

is indicated in adults with severe aortic stenosis when symptoms or
ventricular dysfunction arise, in which surgery replacement of valvular prosthesis is performed (Ross surgery is occasionally conducted in
young people; in adults, the valvulopathy results are negative).
Card iology
Figure 96. Indications for surgical treatment of aortic stenosis

Medical treatment is of limited use in severe aortic stenosis. In general,
surgery is indicated in adults with severe aortic stenosis when symptoms or ventricular dysfunction arise, in which surgery replacement of
valvular prosthesis is performed (Ross surgery is occasionally performed
in young people).
Mechanical prosthesis is indicated in children with severe congenital
aortic stenosis because they have a greater risk of sudden death and
progressive ventricular dysfunction. Results of balloon valvuloplasty in
children are positive, making it the first-choice treatment.
Transcatheter aortic valve replacement is recommended in patients
who meet an indication for aortic valve replacement, who have a prohibitive risk for surgical replacement.
Figure 95. Aortic stenosis auscultation
Aortic valvular sclerosis without stenosis is common in the elderly with

hypertension and produces a murmur with similar characteristics (albeit short and with low intensity). In order to dierentiate them, remember that severe aortic stenosis is usually accompanied by a decrease in
intensity and inverted splitting in S2, and, at times, by parvus et tardus
pulse.
65
11.5. Aortic Regurgitation
Symptoms combine both the eects of low anterograde flow with retrograde congestion.
The most common cause of aortic regurgitation in developed countries

is that associated with bicuspid aortic valve and primary diseases causing dilation of the ascending aorta or the sinuses of Valsalva.
Management of patients with AR is summarized in Figure 98.
Considering etiologies of aortic regurgitation as a

result of direct involvement of aortic leaflets, we
must mention rheumatic heart disease (chronic)
and infective endocarditis (acute).
Considering etiologies of aortic regurgitation
because of ascending aorta dilatation, we
must mention Marfan Syndrome (chronic) and
acute thoracic aortic dissection (acute). LV volume overload (because of blood regurgitation)
usually causes dilation, and, in the long-term,
it may deteriorate systolic function (ejection
fraction).
The characteristic pulse of aortic regurgitation is magnus, celer et altus, and, at times,
bisferiens (two systole peaks). The dierence
between systolic and diastolic pressure is distinctive and produces characteristic physical
signs (Corrigan, Musset, Quincke).
Aortic Regurgitation
Severe AR
(stages C and D)
Vena contracta > 0.6 cm
Holodiastolic aortic
flow reversal
RVol 50%
ERO 0.3 cm2
LV dilation
Progresive AR
(stage B)
Vena contracta 0.6 cm
RVol < 60 mL/beat
RF < 50%
ERO < 0.3 cm2
Other cardiac surgery

Symptomatic
(stage D)
LVEF 50%
LVEF 50%
LVEF < 50% Other cardiac
LVESD > 50 mm LVEDD > 65 mm
(stage C2)
surgery
(stage C2)
Low surgical risk
The diastolic murmur of aortic regurgitation

may be preferentially auscultated in the Erb
accessory aortic focus (third left parasternal
intercostal space) rather than in the aortic
focus. If the regurgitation jet flows to the anterior mitral cusp, it may obstruct opening of
the valve and produce mitral fluttering and,
sometimes, mitral stenosis (Austin-Flint murmur) (Figure 97).
YES
NO
Periodic monitoring
AVR
Asymptomatic
(stage C)
AVR
AVR
AVR
LVEF 50%
LVESD 50 mm
LVEDD 65 mm
Stages of Chronic Aortic Regurgitation (AR):

- Stage C: Asymptomatic Severe AR.
- Stage A: at risk of AR.
- Stage D: Symptomatic Severe AR.
- Stage B: Progressive AR.
Figure 98. General performance procedure for severe aortic regurgitation

Medical treatment includes diuretics for pulmonary congestion. Digoxin may be administered as a positive inotrope. Administration of
vasodilators (ACE inhibitors) is not eective to postpone surgery. Its
use is only recommended in case of ventricular dysfunction, arterial
hypertension, or when surgery is contraindicated. In patients with Marfan syndrome, beta blockers and ACE inhibitors or ARBs must be considered.
Erb focus
(aortic accessory)
Radiates to left
sternal border
In general, surgery is indicated when symptoms appear, when systolic

function deteriorates (LVEF < 50%) or when LV dilates (See Figure
98).
MT
AP
Hyperflux murmur
MT
Austin-Flint murmur
Figure 97. Aortic regurgitation auscultation
66
The presence of ascending aorta dilation along with severe aortic regurgitation implies concomitant operative intervention to repair the aortic
sinuses or replace the ascending aorta in patients with:
Bicuspid aortic valve if the diameter of the aortic sinuses or ascending aorta is greater than 4.5 cm.
Marfan Syndrome when the aorta reached 4.5 cm.
Patients with ascending aorta or aortic sinus diameter 4.5 cm or
greater.
Card iology
Prosthesis replacement is the usual surgical treatment when only the

valve is aected. In some cases it may be possible to repair the aortic valve. In case of dissection or major dilation of the aortic root, either David Procedure (ascending aortic valve is kept and reconnected,
preserving the native aortic valve) or Benthal Procedure (implant of a
valvular tube with reimplantation of coronary arteries when the native
aortic valve is too damaged) may be performed.
The most common cause of respiratory failure is pulmonary hypertension, usually secondary to left heart diseases.
Pulmonary valve disease murmurs are increased during inspiration
(Rivero-Carvallos sign).
Graham-Steeles murmur is the murmur of respiratory failure.
Treatment for severe pulmonary stenosis is usually by means of percutaneous catheter-balloon valvuloplasty.
For severe respiratory failure, an annuloplasty is performed concurrently with surgery on the originating left heart valve disease.
11.6. Right Valvular Heart Diseases
Vascular
Table 52 shows the main characteristics of tricuspid valve disease.
STENOSIS
REGURGITATION
Most frequent
cause
Rheumatic
Functional:
pulmonary
hypertension
Organic: endocarditis
Symptoms
Of associated left-sided
valve disease
Systemic congestion
Of originating
disease
Systemic congestion
Jugular pulse
Increased A wave
Increased V wave
Decreased (reversed)
x sinus
Auscultation
Diastolic murmur
Increase during
inspiration
Systolic murmur
Increase during
inspiration
Severity
Severe: T 1/2 190 ms or

valve area 1.0 cm2
Reversed systolic flow

in suprahepatic veins
Medical
treatment
Low sodium diet, diuretics
Low sodium diet

and diuretics
Surgery
instructions
Severe tricusipd stenosis

(TS) at the time
of operation for
left-sided valve disease
Isolated, symptomatic
severe TS
Recommended for
patients with severe
tricuspid regurgitation
(TR) undergoing leftsided valve surgery
Mild, moderate, or
greater functional
TR at the time of leftsided valve surgery
with either tricuspid
annular dilation or
prior evidence of right
heart failure
Symptoms because
of severe primary
TR unresponsive to
medical therapy
Technique
Bioprosthesis
Commissurotomy
Percutaneous
valvuloplasty in some
cases
Prosthetic
De Vega
annuloplasty
Bioprosthesis
Table 52. Main features of tricuspid valve disease

With regard to pulmonary valve disease, it should be noted that:
Pulmonary stenosis is most frequently congenital.
Chapter 12
Disease
12.1. Aortic Aneurysm

Infrarenal abdominal aortic aneurysm is the most common form, although they may occur in almost any artery (Figure 99).
Figure 99. Types of vascular aneurysms

Infrarenal abdominal aortic aneurysm (IAAA) is most frequent among
males. The most frequent etiology is atherosclerosis. The aneurysm is
67
often identified by chance during another addominal test. The main risk
factors for IAAA rupture are diameter, hypertension, active smoking, female sex and presence of symptoms (expanding aneurysm).
Ascending aorta thoracic aneurysm may appear because of cystic medial necrosis (typical with Marfan, Ehler-Danlos syndromes or associated
with bicuspid aortic valve) or tertiary syphilis.
Abdominal aortic aneurysm diagnosis is usually by means of abdominal ultrasound. However, in case of suspected complex aneurism or in
view of surgical intervention, CT angiography or magnetic resonance
angiography with contrast are more useful techniques. Opaque contrast aortography may underestimate the aneurysms diameter in
case of mural thrombus, but it is excellent for assessing adjacent arterial branches. Currently, an aortography is not usually required for
intervention, since CT and magnetic resonance angiography may provide sucient and accurate preoperative information.
Examination of these patients is key for the associated coronary disease; the latter may aect the prognosis and surgical risk of the patient.
Current treatment for this kind of aneurysms is shown in Figure 100.
Suspected abdominal
aorta aneurysm
Figure 101. Ascending aorta aneurysm

Ultrasound screening
for detection.
CT or MR angio
for detailed
characterization
Symptomatic
Asymptomatic
CT Angio
Consider:
RM Angio
Aortography
*Emergency
surgery
No smoking
Treat risk factors
Consider -blockers
> 5.5 cm
Low
surgical
risk
5.0-5.5 cm
High
surgical
risk
Programmed If not
feasible
surgery
Consider
intervention
if low surgical
risk
Consider
graft stent
4.0-5.5 cm
Follow-up every
6 to 12 months
using ultrasound
or CT scan
Growth
> 0.5 cm/year
*Preferrable
if there
is no rupture
Figure 100. Management of abdominal aortic aneurysms

The presence of symptoms, growth velocity over 0.5 cm a year, or the
presence of aorta diameter over 5.5 cm (5 cm in patients with low surgical risk) is indicative for surgery.
Surgical repair requires a midline transabdoiminal approach or an extraperitoneal incision in the left flank to perform aneurysmectomy and
placement of a synthetic tubular graft with iliac extension (if the territory is also aected). Endovascular repair clearly represents a major
advance in patients who have severe cardiopulmonary disease or other
risk factors.
Aneurysms involving the aortic arch or descending aorta usually have
atherosclerotic etiology (Figure 101).
68
Treatment for thoracic aortic aneurysms is shown in Figure 102.

A diameter of ascending aorta aneurysms 5.5 cm, the presence of symptoms, or a growth velocity 0.5 cm a year are indications for surgery. In
case of association with bicuspid aortic valve, aortic dissection or Marfan
syndrome, the former criteria are applied with lower diameters (4-5 cm)
according to the presence of other associated rupture risk factors.
For patients undergoing aortic valve surgery coexisting with ascending
aorta dilation, aorta intervention is also recommended at lower diameters (4.5 cm). Bentall procedure is used for ascending aorta aneurysms
with associated aortic failure; it consists of implanting an aortic valve
graft with reimplantation of coronary arteries.
Hypertensive treatment of choice in Marfan patients consists of beta
blockers and possibly ARB.
The most frequent type of peripheral aneurysm is popliteal. It is usually
bilateral and, if it becomes thrombotic, there is a high risk of losing the
limb involved. Therefore, treatment requires surgical repair based on
aneurysm exclusion and placement of femoral popliteal bypass.
12.2. Acute Aortic Syndrome

Traumatic aortic rupture usually occurs after car accidents or serious
falls and is often located distal to left subclavian artery.
Acute aortic syndrome comprises three entities: classic aortic dissection, intramural aortic hematoma and penetrating aortic ulcer. Clinical
and therapeutic aspects of these three pictures are nearly the same.
The main characteristics of intramural hematoma and penetrating ulcer
are shown in Table 53.
Card iology
Thoracic
aortic aneurysm
Symptomatic
Asymptomatic
Emergency surgery
With high surgical
risk, consider graft
stent in descending
aorta
Ascending
High risk factors of rupture

or dissection:
Connective tissue disease: Marfan,
Ehler Danlos, Loeys-Dietz....
Bicuspid aorta, dissection, Turner,
Noonan...
Family association
Semi-annual size
follow-up
Treat risk factors
Avoid intense exercise
Beta blockers
Arch
Descending
Surgery
if > 5.5 cm
Surgery
if > 6 cm*
* With connective
tissue disease,
consider graft
stent if > 5.5 cm
* If high surgical risk,
consider graft stent
Yes
No
Consider surgery
if > 4-5 cm
Surgery
if > 5.5 cm
or growth
> 0.5 cm/year
Figure 102. Procedure for action in the event of thoracic aortic aneurysm
INTRAMURAL AORTIC
HEMATOMA
Etiopathogenesis Adventitia bleeding
towards the media
More frequent
in descending aorta
PENETRATING ULCER
Intima disruption
secondary
to atherosclerotic
damage
More frequent in
descending aorta
Epidemiology
Elderly patients with same risk factors as aortic

dissection
Diagnosis
CT angio, MR angio or TEE focus on thoracic aorta
Treatment
Similar to aortic
dissection
in the corresponding
segment of the aorta
Similar to type B
dissection
Surgery if:
- Hemodynamically
unstable
- Pseudo aneurysm
- Acute rupture
- Continuous pain
- Distal embolization
Table 53. Intramural hematoma and penetrating ulcer

Hypertension is the main condition associated with increased wall stress
and development of thoracic aortic dissection. The dissection flap is commonly found in the ascending thoracic aortic, within its first 2-3 cm.
Acute aortic dissection must be considered in all patients presenting
intense chest, back or abdominal pain, especially with migratory features. Dissection may involve arterial branches and lead to ischemia
perfusion deficits in the area involved. Typical findings of aortic dissection are asymmetric decrease of upper limb pulses and a diastolic
murmur of aortic valve regurgitation.
Stanfords classification (Table 54) divides acute aortic syndromes (and
aortic dissection) into type A or proximal: if the ascending aorta is involved (the most frequent, prognosis is poor, and requires surgical repair) and type B or distal: if they do not involve the ascending aorta (less
frequent, better prognosis, treatment is medical and it should be managed medically unless life-threatening complications develop).
Urgent and definitive imaging of the aorta using transesophageal echocardiogram, computed tomographic imaging, or magnetic resonance
imaging is recommended to identify or exclude thoracic aortic dissection in patients at high risk for the disease. Figure 103 shows recommendations for acute aortic syndrome management.
DE BAKEY CLASSIFICATION
I
Ascending aorta rupture, extending to descending

aorta
II
Ascending aorta rupture (only)
III
Descending aorta rupture

STANFORD CLASSIFICATION
A (proximal)
Involving ascending aorta
B (distal)
Not involving ascending aorta
Table 54. Aorta dissection classication
69
blockers or labetalol. Type A dissections require emergency surgical repair

(Figure 104). Anticoagulants are formally contraindicated.
Figure 105. Surgical treatment of type-A dissection
12.3. Deep Vein Thrombosis

Deep vein thrombosis (deep vein thrombophlebitis) is the most frequent cause of pulmonary embolism. Predisposing factors are blood
stasis, hypercoagulability and traumatism (Virchows triad).
Figure 103. Aortic dissection classication
Blood pressure and heart rate should be controlled in all patients with
acute aortic syndrome, usually with nitroprusside and intravenous beta
Patients undergoing orthopedic-traumatologic treatment either surgical or leading to immobilization are at risk of suering deep vein thrombosis. The main causes are shown in Table 55.
Diagnosis is usually confirmed by means of
Doppler ultrasound. Treatment consists of
rest, elevation of involved limbs to avoid edema, and anticoagulation to avoid progression
and development of pulmonary embolism.
Anticoagulation therapy must be commenced
and maintained for three months. However,
patients with recurrent episodes or nonreversible causes must be considered for indefinite anticoagulation.
Acute aortic syndrome treatment
Suspected
CT Angio + TEE
Medical
treatment
Pain and signs/symptoms typical of hypertensive patients

Mediastinal widening
Consider MR angio if stable

Conduct TEE if there is aortic insufficiency or too unstable to transfer
Opiates. Avoid anticoagulation

Beta blockers or calcium antagonists: target HR < 60 bpm
Vasodilators: ACE inhibitors or nitroprusside (if systolic BP > 120 mmHg)
General management of this entity is shown

in Figure 106.
Type A
Type B
High risk data:

Hemodynamically unstable
Fast growth
Peri-aortic hematoma
Ischemia in arterial branches
Urgent surgery
Consider after stabilization (24-72 hours)
only with
- Stable retrograde thrombosis dissection
- Stable intramural hematoma with aorta
< 50 mm and hematoma < 10 mm
Contraindicated with irreversible brain damage
Consider medical management if:
- Chronic dissection
- Stable and limited to arch
Yes
No
Consider graft stent
Close follow-up
* Fenestration in some cases
Figure 104. Management of acute aortic syndrome
70
Complete early ambulation, pneumatic compression stockings, and low doses of anticoagulants are used for prophylaxis of deep vein
thrombosis.
Other Venous Disorders

Chronic venous insuciency is usually the consequence of deep vein thrombosis, since the
healing process may cause a dysfunction of
the vein valve system, which leads to blood reflux to the superficial venous system through
perforating veins (Figure 107).
Card iology
RISK OF DEEP VEIN THROMBOSIS

Immobilization (paralysis, stroke-derived sequel, orthopedic
treatments, long flights: economy-class syndrome and others)
Surgery: especially major orthopedic surgery of lower limbs (hip
surgery represents a major risk factor for DVT, which may appear
in up to 50% of cases) and abdomen
Admission for medical reasons
Previous history of DVT or pulmonary embolism
Pregnancy, postpartum, hormone-based therapies
Heart failure or myocardial infarction
Obesity
Congenital or acquired hypercoagulability: antithrombin III
deficiency, protein C and S deficiency, antiphospholipid syndrome,
nephrotic syndrome, activated protein C resistance (Factor V Leiden)
and dysfibrinogenemia
Neoplasm: pancreas, ovary, lung, breast and others. Sometimes
they are associated with surface thrombosis or migrating
thrombophlebitis as paraneoplastic syndrome (e.g. Trousseau
syndrome)
Intravascular (venous) devices or catheters, both transient
and permanent (pacemakers, defibrillators, central catheters
for chemotherapy or hemodialysis)
Normal
venous flow
Competent closed valve
Incompetent open valve.

Retrograde flow
Table 55. Risk factors of deep vein thrombosis (DVT)
Normal vein
Varicose vein
Figure 107. Venous valve system incompetence

Venous ulcers usually aect the internal
malleolus and have a humid base. They are
less painful than arterial ulcers, Figure 108.
Posthrombotic syndrome leading to chronic venous insuciency can be treated using
elastic compression stockings, appropriate
management during the acute phase of
deep vein thrombosis, phlebotonic drugs
(discrete ecacy) and avoiding prolonged
standing.
Superficial venous thrombosis, unlike the
deep form, does not cause pulmonary embolism or chronic venous insuciency. Its
treatment consists of relative rest, elastic
compression stockings and anti-inflammatory drugs. It does not require oral anticoagulation.
Varicose veins may be a consequence of
chronic venous insuciency after a deep
vein thrombosis, weakness of the venous
valve system, often with family history,
may also cause varicose veins. When the
problem aects small areas, it only represents an esthetic issue, but when large
areas are aected, truncal varicose veins
may require surgical intervention (phlebectomy).
Figure 106. Overall management of deep vein thrombosis (DVT)
The most common cause for superior vena

cava obstruction is tumors, especially microcytic lung tumors, which are not resectable.
It may cause a typical edema on the face and
upper limbs (Figure 109).
71
Figure 108. Dierential treatment between venous and ischemic ulcers

Figure 110. Atherosclerosis location
Depending on the location of the artery obstruction there are three
clinical forms: Leriches syndrome or aorto-iliac occlusive disease (claudication in leg, buttocks and, in men, usually erectile dysfunction), popliteal artery occlusive disease (the most common clinical type in which
pain of intermittent claudication is most often in the calf) and tibial and
peroneal occlusive disease (Figure 111).
Figure 109. Patient with edema on the face and upper limbs
12.4. Peripheral Artery Disease

Peripheral artery disease, also known as chronic limb ischemia, is due
to atherosclerosis and mainly aects the lower limbs of elderly patients,
causing intermittent claudication symptoms.
The presence of chronic ischemia in lower limbs is detected by the presence of ankle-brachial index below 0.9.
The most frequent place for atherosclerotic obstruction in the lower
limbs is the adductor canal or Hunters canal, located in the thigh (it
thus causes femoral and popliteal claudication; Figure 110).
72
Figure 111. Clinical forms of chronic arterial occlusion according

to location of lesion
Medical treatment consists of adopting hygienic and dietary steps

(most importantly, smoking cessation and doing adapted physical exercise), chronic antiplatelet treatment (clopidogrel appears to be slightly
better than ASA in absolute terms), statins, ACE inhibitors (although
they do not improve claudication, they lower the risk of cardiovascular
episodes) and, sometimes, drugs that improve walking distances until
claudication, especially cilostazol (or, less markedly, pentoxifylline).
Card iology
Intermittent claudication
Arterial revascularization is recommended in patients with incapacitating

intermittent claudication, pain at rest or with ischemic ulcers... (stages IIb,
III and IV, respectively, of Fontaine classification; Table 56).
STAGE I
Cold, tingling, paresthesia, cramps, skin pallor,

appendages disorders (nails, hair)
STAGE II
Intermittent claudication:
IIa: nonincapacitating intermittent claudication
(for patients regular activity)
IIb: incapacitating intermittent claudication
(for patients regular activity)
STAGE III
Pain or paresthesia at rest
STAGE IV
Ischemic ulcers (ulcers, gangrene)
Aprox. Mortality
20% to 30% in five years
Prognosis
of involved limbs
Stable
75%
Advanced
claudication
aprox. 15%
Need
of surgery
aprox. 10%
Amputation
5%
Coronary disease
Cardiovascular
disease
Figure 113. Five-year prognosis with intermittent claudication
Table 56. Clinical Fontaine stages

Aortoiliac surgical treatment of choice is aorto-iliac anatomic bypass or
unilateral or bilateral aortofemoral bypass. In cases with high surgical
risk, extra-anatomy axillary-femoral or femoro-femoral crossover bypass are the alternatives used.
For stenosis in the iliac artery with incomplete occlusion, percutaneous
treatment by means of angioplasty oers good results and is usually the
first option.
In the femoro-popliteal sector, stenosis is usually too long and therefore percutaneous procedures often have worse results, limited to
nonocclusive short stenosis of the superficial femoral.
Figure 114. Anatomy of thoracic outlet of upper limb nerve bundle
Here, the treatment of choice is anatomic bypass, using autologous saphenous vein grafting.
General treatment of this disease is shown in Figure 112.
Etiology should be dierentiated since local thrombosis may cause the

same symptoms. Table 57 shows data which enables making a dierential diagnosis.
Acute arterial ischemia treatment is shown in Figure 115.
Acute peripheral occlusion

Acute
Peripheral embolism
Suspected acute arterial thrombosis
No vascular history
Normal contralateral pulse
History of claudication
or vascular disease
Embolic heart
disease
Nonembolic
heart disease
Arteriography
Poorly-tolerated ischemia
(with motor and sensitive
changes)
Figure 112. Examples of extra-anatomy bypass in proximal area of lower
Well-tolerated ischemia
(with no motor
or sensory changes)
limbs
Long-term prognosis of the disease is shown in Figure 113.
Other Artery Diseases

The most common etiology of acute arterial ischemia is an embolism due to
atrial fibrillation and its treatment of choice is embolectomy (Figure 114).
Consider
Fibrinolysis
Surgery
Consider
if high risk
Figure 115. Acute arterial occlusion protocol for treatment
73
ARTERIAL EMBOLISM
ACUTE ARTERIAL THROMBOSIS
Anamnesis
Embolic heart disease (AF)
Lower limb chronic ischemia history

(prior intermittent claudication)
Obliterating atherosclerosis risk factors
Rare
Frequent
Onset of symptoms
Sudden/Acute
Sudden/progressive (acute/subacute)
Predominant symptom
Pain
Pain/Paresthesia (+++)
Lower limb chronic ischemia findings
Rare
Frequent (decrease or absence of arterial pulse in contralateral limb)
Arteriographic findings
STOP image (cupola)

Minimum atherosclerosis
Scarce collateral circulation
Irregular STOP image

Segmented arterial atherosclerotic lesions
Abundant collateral circulation
Table 57. Dierential diagnosis between stroke and thrombosis

Buergers disease or thromboangiitis obliterans is strongly connected with
smoking and causes ischemic intermittent claudication in distal areas, Raynauds phenomenon and migrating thrombophlebitis. The main dierences
with obliterating arterial atherosclerosis are shown in Table 58.
OBLITERATING
ATHEROSCLEROSIS
OBLITERATING
THROMBOANGIITIS
Age
Usually, over
50 years of age
Young, below
45 years of age
Blood pressure
Usually, hypertension
Often hypotension
Raynauds syndrome No
and migrating
phlebitis
Commonly present
Cholesterolemia
High
Normal or low
ESR (erythrocyte
sedimentation rate)
Normal
High
X-rays,
arteriography
Hard, calcified,
deformed, tortuous,
irregularly-sized
arteries
Atheromatous aorta
Straight and smallcaliber arteries, with

no calcification
Nonatheromatous
aorta
Table 58. Dierential diagnosis between atherosclerosis and obliterating

thromboangiitis
Quitting tobacco usually stops the progression of the disease; therefore, it is the most important step. The appearance of new lesions is a
sign that patient has not achieved cessation.
Administration of nicotine gum or patches may be sucient to maintain
the disease active. In certain cases, deviation of large vessels and local
debridement of lesions may be performed. Anticoagulant therapy has
not yielded the expected results and use of corticosteroids has not been
useful either. In some cases, amputation is necessary, usually more limited than with obliterating atherosclerosis.
In subclavian steal syndrome, the occlusion is in the subclavian artery
before the exit of the vertebral artery and causes symptoms of vertebrobasilar insuciency (Figure 116).
Raynauds phenomenon consists of recurrent episodes of vasoconstriction
aecting fingers most commonly, caused by exposure to cold or intense
emotions. It is important to dierentiate Raynauds disease from Raynauds
phenomenon. Raynauds disease is not the result of an underlying associated
medical condition (idiopathic); it comprises more than half the cases, being
predominant among young women and with usually milder manifestations.
74
Figure 116. Subclavian steal syndrome

On the contrary, Raynauds phenomenon may be secondary to an underlying disorder (Table 59), often some process causing organic lesions of the arterial wall.
RAYNAUDS DISEASE
Idiopathic
CONNECTING TISSUE
DISORDERS
BLOOD DYSCRASIA
TRAUMA
DRUGS
NEUROLOGIC
ALTERATIONS
Scleroderma
Systemic erythematosus lupus
Rheumatoid arthritis
Diabetes mellitus
Dermatomyositis
Cryoglobulinemia, macroglobulinemia
of Waldenstrom, myeloproliferative disorders
Hammer syndrome
Vibration lesions
Electric shocks
Ergotamine
-blockers
Bleomycin, vincristine, cisplatin
Syringomyelia
Carpal tunnel syndrome
Poliomyelitis
Table 59. Diseases where Raynauds phenomenon is involved
Card iology
Most patients with mild and infrequent episodes do not require special treatment. They should be instructed to avoid cold environmental
temperatures by using thick gloves and socks, and to avoid mechanic
microtrauma. Tobacco is contraindicated due to its potential vasoconstricting eect. In severe cases, vasodilator therapy with drugs may be
administered, calcium antagonists are advisable to this end, and if there
is no response, surgical sympathectomy may be considered, although
any benefits are usually temporary.
lympho-venous anastomosis to rechannel the flow from an obstructed

lymphatic vessel to the venous system.
Syncope
Chapter 13
12.5. Lymphedema
The most common cause of lymphedema globally, and mostly in tropical Africa, is filariasis, which causes lymphatic obstruction due to nematodes and a corresponding inflammatory response, which leads to elephantiasis.
Frequent causes of lymphedema are tumors, both due to lymphatic
infiltration of malignant cells and treatment (surgery with lymph
node exeresis or fibrosis caused by radiation therapy to the lymph
system).
The clinical aspects are very important for diagnosis of this disease,
since lymphography is rarely used. Lympho-gammagraphy may be used
to detect the blocking point of lymphatic drainage, and it is a useful
technique in oncology to detect the sentinel ganglion where sick tissue is drained and in order to plan the surgery.
Lymphedema, unlike edemas with dierent etiology, has an orange
skin appearance on the surface area involved, it has a hard feel
(woody) and shows little response to diuretics or rest. In secondary
lymphedema, ducts are usually dilated and it is possible to determine
the occlusion level. On the contrary, in the primary form, lymph vessels
are absent or are hyperplastic or ecstatic.
The main goals of lymphedema treatment are to control edema
(superficial lymphatic massage, soft, and applied from a distal to
proximal direction, physiotherapy and other techniques; any element compressing the limbs, venous punctures, intense exercise
or trauma of the involved limb should be avoided, which should
be as long as possible above the heart level), maintain a healthy
and clean skin, and prevent complications such as cellulitis or erysipelas and lymphangitis. It is advisable to raise the feet while in
bed, use compression stockings and bandages after emptying the
edema by means of manual drainage; or use sequential pneumatic
compression boots during the day. Especially with lymphedema after breast surgery, good results have been shown with low-level
laser therapy.
Syncope is a symptom which consists of transient loss of consciousness

(usually just a few seconds, sometimes for some minutes), associated
with postural tone loss and falling, with spontaneous and complete recovery, because of transient global cerebral hypoperfusion. There may
be warning signs (weakness, dizziness, ear ringing, cold sweat). Presyncope is the imminent feeling of syncope without actually losing consciousness.
13.1. Differential Diagnosis

Since there is no transient loss of consciousness, casual falling, cataplexy (loss of muscle tone associated with emotions or fits of laughter,
frequently associated with narcolepsy), drop attacks (blue knees disease in middle-aged women), psychogenic pseudosyncope or transient
ischemic attack, are not true syncope episodes. Even with transient loss
of consciousness, cases of cranio-encephalic trauma, epilepsy, intoxication, and narcolepsy, loss of consciousness with endocrine and metabolic origin (hypoglycemia, hypoxia or hypocapnia because of hyperventilation) or vertebrobasilar transient ischemic attack are not syncope
episodes either. In subclavian steal syndrome, syncope is exceptional
without other associated focal vertebrobasilar symptoms, triggered by
physical exercise of the ipsilateral arm with obstruction.
True syncopes are classified into three groups, according to their pathophysiology (Table 60).
Clinical history is the most profitable data for diagnosing the type of
syncope. It is essential to investigate the situation and triggering factors, symptoms before and after the episode, as well as the presence of
heart disease or neuropathy by means of thorough physical examination. Episodes during exertion, associated palpitations, episodes while
lying down, or family history of sudden death are risk data.
The presence of redness, pain and edema usually involves cellulitis or

bacterial lymphangitis (it is known as linear swelling throughout the
length of the limb). Common etiologic organisms are positive-coagulase staphylococcus or -hemolytic streptococcus, which require strong
treatment, nearly always with intravenous antibiotics. Chronic lymphedemas may exceptionally progress to lymphangiosarcoma.
Among other complementary tests, electrocardiogram (ECG) (if normal,

it almost completely rules out cardiac origin of syncope), orthostatism
test or active standing (measurement of BP in supine position and after three minutes of standing, a drop of over 20 mmHg of systolic BP
or over 10 mmHg of symptomatic diastolic BP is considered positive),
carotid sinus massage (which is diagnostic if it reproduces the syncope
with a pause over three seconds or with a BP drop over 50 mmHg), basic
blood work and chest X-rays are key.
Surgery is used in severe cases which do not respond to medical treatment and it consists of removing hypertrophied tissue and applying
There are some ECG alterations that indicate a high risk in case of syncope (Table 61).
75
REFLEX OR NEURALLY MEDIATED SYNCOPE

Vaso-vagal (neurocardiogenic) classic: induced by emotional stress,
fear, blood-phobia, pain or orthostatic stress
Situational syncope: tusigen, sneezing, gastrointestinal stimulation
(deglutition, defecation, abdominal pain, glossopharyngeal neuralgia),
postprandial, postexercise, playing wind instruments, laughter
Carotid sinus syncope
Atypical forms
emotional stress. It appears to be caused by a reflex alteration which

usually begins with a decrease in venous return and secondary release
of catecholamines which cause strong ventricular contractions, with a
relatively empty ventricle, with associated vagal discharge, hypotension
and bradycardia. It has an excellent prognosis with almost null mortality.
ORTHOSTATIC HYPOTENSION SYNCOPE

Primary autonomic failure (Shy-Drager, Parkinson, Lewy bodies
disease, etc.) or secondary (neuropathy, diabetes, amyloidosis,
uremia, marrow damage, etc.)
Drugs (diuretics, vasodilators, neuroleptics, antidepressants) or alcohol
Volume depletion (bleeding, Addison, dehydration, digestive loss)
CARDIAC SYNCOPE
Of arrhythmic origin:
- Sinus diffusion
- AV blocks
- Pacemaker or defibrillator dysfunction
- Supraventricular or ventricular tachyarrhythmia
- Pro-arrhythmic drugs
Cardiopulmonary structural disease: heart valve disorders,
myocardial ischemia, pulmonary embolism, hypertrophic
cardiomyopathy or other outflow tract obstructions, aortic
dissection, pericardium tamponade, atrial myxoma, valve prosthesis
dysfunction, pulmonary hypertension
Table 60. Syncope pathophysiology types
RHYTHM
ALTERATIONS
QRS ALTERATIONS
REPOLARIZATION
ALTERATIONS
2.nd or 3.rd degree AV block

Sustained or nonsustained ventricular
tachycardia
Pre-excitation
Branch block
Left-sided ventricular hypertrophy criteria
Pathologic Q waves
Epsilon wave
Right precordial negative T waves

Ischemic changes
Brugada pattern
Long QT interval
Table 61. ECG Alterations involving high risk in patients with syncope
If this initial assessment is enough to reach a diagnosis, patient
treatment follows. If not, it is known as syncope of unknown origin,
and other tests are necessary that should be considered only with
certain suspected clinical origins, such as Holter, echocardiography,
tilt test, electrophysiology study, stress test, cardiac catheterization
catheterization. Events loop recorder or implantable Holter are useful with syncope of unknown origin with suspected arrhythmic origin, especially when the frequency of syncope is very low (since in
this context it is rare for the conventional Holter to provide any data
of interest).
The figure below shows the general treatment of a patient with syncope
(Figure 117).
Vaso-vagal syncope is the most common type. It is triggered by seeing blood, prolonged standing, hot or tight environments, or even by
76
1. Confirmation of Suspected Syncope

Was there loss of consciousness?
No
Fall, tonal crisis, cataplexy
Yes
Was recovery spontaneous?
No
Metabolic alteration,
intoxication, RPC
Yes
Is it due to global brain hypoperfusion?
No
Concussion, epileptic crisis,

pseudosyncope,
TIA/vertebrobasilar migraine
Yes
2. Risk Classification of Syncope

Syncope during exertion?
Syncope while sitting?
Syncope with severe trauma?
Yes
Cardiogenic profile syncope
No
Suspected neuromediated
or orthostatic syncope?
Yes
Benign Syncope
No
Syncope with Unknown Profile
3. Patient Risk Classification

Family History:
Sudden death
Family heart disease
Structural Heart Disease
(history, PE, Chest X-ray +/- Echo)
Yes
High Risk
No
Low Risk
Abnormal ECG
Figure 117. Clinical Approach to Patient with Potential Syncope

A tilt test or tilt table test (not required for diagnosis in most cases)
enables dierentiating cardioinhibitory (with predominant bradycardia), vasodepressor (with predominant hypotension) or mixed forms.
Treatment consists of explaining the benign quality of the clinical picture in spite of recurrence risk, advising the avoidance of triggering
factors and, in patients with prodrome symptoms, isometric counterpressure maneuvers (cross legs while standing). It may be useful to
increase hydrosaline intake, avoiding diuretics and vasodilators. In refractory cases, drugs may be used, drugs may be used and midodrine
is the drug of choice (adrenergic receptor stimulants), since serotonin
reuptake inhibitors, other adrenergic stimulants (etilefrine), and mineralocorticoid drugs (useful with orthostatic syncope), are not usually
eective and show common side eects. Beta blockers are currently
considered contraindicated.
In very special cases of vaso-vagal syncope recurrent in patients over
40 years of age, with no response to usual treatment and with a cardioinhibitory form, the use of a pacemaker may be prescribed, the same
Card iology
as with cardioinhibitory carotid syncope, although this does not always

decrease syncope episodes since it does not mitigate the vasodepressant eect.
Details for other causes of syncope are shown in various sections of this
manual.
The main types of shock are (Figure 118):
Hypovolemic shock
Obstructive shock
Diaphoresis
Pneumothorax
Vomiting
Miscellaneous
Burns
Pulmonary embolism
Chapter 13
Tamponade
Bleeding
Cardiogenic
shock
14.1. Shock
Shock is a syndrome characterized by a decrease in tissue perfusion
below its metabolic demands. If the situation persists, organ dysfunction and tissue damage may be observed. Generally, compensation
mechanisms are activated (increase in adrenergic tone, increase in adrenergic tone, contractility and heart rate and with cutaneous, muscular and splanchnic vasoconstriction) to preserve vital organs (central
nervous system and heart). However, if these mechanisms extend in
time, they may be deletereous (Table 62).
TYPES OF SHOCK
VENOUS
CENTRAL
PERIPHERAL
CARDIAC
OXYGEN
VENOUS
VASCULAR
OUTPUT
SATURATION
PRESSURE
RESISTANCES
(%)
Hypovolemic
Cardiogenic
Obstructive
Septic Hyperdynamic
Hypodynamic
or late
Neurogenic
Anaphylactic
Table 62. Hemodynamic patterns for the main types of shock

Shock is not a synonym for hypotension, considering low blood pressure
(BP) figures may suce to maintain the proper blood supply for the tissues if certain compensation mechanisms are activated.
Therefore, diagnosis of shock is clinical and requires three conditions:
1. Arterial Hypotension
2. Tissue Hypoperfusion: cold and paleness of limbs with spots, slow
capillary refill in nail beds, and secondary metabolic acidosis caused
by lactate accumulation.
3. Organic dysfunction: of the central nervous system with a decrease in the level of consciousness, of the kidney (with oliguria levels
lower than 0.5 mL/kg/h), impaired breathing or myocardial ischemia.
Distributive
shock
Intense internal
diuresis
Figure 118. Types of shock

Hypovolemic is the most common. It is caused by a decrease in the
blood volume available in the interior of the vessels, due to bleeding, dehydration and third space sequestration.
Intrinsic cardiogenic shock caused by a decrease in cardiac output
because of lack of cardiac systo-diastolic function. The most common cause is extensive acute myocardial infarction.
Extracardiac cardiogenic or obstructive/compressive shock caused
by extrinsic compression of cardiac cavities resulting in diastolic
heart failure, as is the case with pericardial tamponade, tension
pneumothorax, large diaphragmatic hernias, mechanic ventilation,
and massive pulmonary embolism.
Distributive shock is characterized by a deficient vascular distribution of cardiac output, which may even be high. The most common subtype of distributive shock is septic shock, which is generally caused by infections in immunosuppressed individuals or by
particularly virulent pathogenic agents found in immunocompetent patients. Septic shock can have two hemodynamic patterns
according to the evolution stage: an initial or hyperdynamic pattern and another in advanced stages or hypodynamic. Neurogenic
shock, another distributive shock subtype, results from severe
damage in the central nervous system such as traumatisms, spinal cord injury, and rachianesthesia and produces an alteration to
sympathetic vessel constrictor tone and a decrease in cardiac output. The remaining distributive shock subtypes are anaphylactic
shock triggered by allergens and activated by an intense release of
vasodilator substances, such as histamine; and finally, toxic shock,
77
linked to intoxication caused by barbiturates, phenothiazines, or

shock connected with certain endocrine disorders such as acute
adrenal insuciency.
Each type of shock has a dierent hemodynamic pattern, facilitating a dierential diagnosis. Frequently, especially in advanced
stages, patients may simultaneously undergo many forms of shock
with opposing hemodynamic patterns, which hinders diagnosis and
treatment (for example, septic shock is often linked to hypovolemic
shock, so that the treatment of the former involves the treatment of
the latter).
Treatment is meant to correct the specific triggering factor and involves using general life support measures depending on the symptoms (artificial respiration, volemic reposition, active vessel drugs,
among others).
14.2. Rheumatic Fever

Rheumatic fever (RF) is a multisystemic autoimmune disease, secondary to pharyngitis, caused by Streptococcus type A (S. pyogenes),and
aecting joints, the skin and subcutaneous cellular tissue, the central
nervous system and the heart (which determines the severity of the disease). Even though RF impact has diminished drastically in the occidental countries, it still constitutes a serious health issue in underdeveloped
countries. RF is seen in children between 5-15 years of age and vascular
sequels are often observed between the ages of 25-40. It is twice more
frequent in women.
Diagnosis is based on Jones criteria (Table 63). At least two major criteria are needed, or one major and two minor criteria, as well as serologic
or bacteriologic data signaling recent streptococcal infection.
MAJOR CRITERIA
Polyarthritis
Erythema marginatum
Subcutaneous nodules
Carditis
Chorea minor
MINOR CRITERIA
Fever
Arthralgia
Previous episode of FR
Previous episode of rheumatic
carditis
Elevated ESR or CRP
Prolonged PR interval
Anti-inflammatory treatment is important, acetylsalicylic acid is generally administered. Arthritis usually resolves within 24-48 hours.
Glucocorticoids are used only if NSAIDs are not enough or if there
are signs of carditis with moderate or severe heart failure.
It is not advisable to start treatment with anti-inflammatory drugs
until the diagnosis of rheumatic fever is clear. Arthralgias can be
treated with non anti-inflammatory analgesics while waiting to see
if the Jones criteria are met not to overestimate a rheumatic fever
diagnosis. Anti-inflammatory drugs are administered for several
weeks after normalization of ESR and C-reactive protein levels, followed by a gradual withdrawal. ASO levels take six months to decrease after initiation of treatment.
In cases of chorea minor, sedatives (especially diazepam) and rest
are very useful. Prolonged bedrest is not recommended, except in
cases of active carditis and persistent or severe heart failure.
Improvement of living conditions, early diagnosis and treatment of the
pharyngeal streptococcus infections are the most important factors for
the prevention of rheumatic fever.
Secondary prophylaxis is performed with an intramuscular injection of
benzathine penicillin G, administered on a monthly basis. Penicillins administered orally, erythromycin and sulfadiazine are a second option.
The last two can be used when the patient is allergic to penicillin.
The latest recommendations in regard to prophylaxis duration are presented in Table 64.
DURATION
CATEGORIES OF PATIENTS
Rheumatic fever without carditis
Five years since the last episode

or until 21 years of age
(the longest period)
Rheumatic fever with carditis,

but no residual valvular disease
10 years since the last episode

(the longest period)
Rheumatic fever with persistent

valvular disease evidenced
by clinical or echocardiographic
signs
10 years since the last episode

(the longest period). Occasionally,
for life
Table 64. Duration of prophylaxis of new episodes of rheumatic fever
14.3. Myocarditis
EVIDENCE OF PRE-EXISTING STREPTOCOCCAL INFECTION

ASO and other anti-streptococcal antibody
Pharyngeal swab culture positive for type A streptococcal
Recent episode of scarlet fever
Table 63. Jones criteria

Antibiotics must be administered immediately for its treatment. Oral
penicillin V (phenoxymethylpenicillin) is recommended for adults, or
benzathine benzylpenicillin (one single intramuscular injection). Amoxicillin is an alternative. If allergic to penicillin, erythromycin can be administered.
During the acute phase, rest is advisable until laboratory abnormalities
disappear (normalization of acute phase reactants).
78
Myocarditis is an inflammation of the myocardium that may be caused

by:
Infectious agents: in nearly half the cases, it is caused by coxsackievirus B (its capsid proteins are similar to actin), but it can also be
caused by other virus, for example adenovirus, echovirus, and arbovirus, influenza.
Hypersensitivity reactions, such as acute rheumatic fever.
Other agents, physical factors - radiation - and chemical factors drugs and toxic agents -.
Primary myocarditis occurs when an etiologic agent is not detected.
A viral etiology or a virus-induced autoimmune response is assumed.
Myocarditis can be fulminant, acute, sub-acute or chronic.
Card iology
Symptoms are nonspecific, sometimes it is dicult to distinguish myocarditis from an acute coronary syndrome or acute pericarditis. Chest
pain is usually similar to pericardial pain due to frequent association
with viral pericarditis (myopericarditis), whose symptoms are more
prominent. However, when there is myocardial involvement, markers
for myocardial damage are elevated. During the previous days, there
may have been a nonspecific viral infection of the upper respiratory
tract or gastrointestinal tract. In some cases, myocarditis produces signs
and symptoms of heart failure because of ventricular dysfunction secondary to inflammatory infiltration of the myocardium and to microvascular damage, as well as arrhythmias that may cause sudden death,
especially in pregnant women and infants. It can also evolve into dilated
cardiomyopathy.
ventricular assist devices and, in some cases, even heart transplantation.
I.
II.
CLINICAL CRITERIA
Acute chest pain (usually pericardial)

Recent onset (last three months)
or worsening of dyspnea with
or without signs of heart failure
III. Subacute/chronic (more than three
months) or worsening dyspnea with or
without signs of heart failure
IV. Palpitations and/or arrhythmias,
syncope or sudden unexplained death
V. Unexplained cardiogenic shock
I.
Physical examination usually reveals nonspecific signs, such as gallop,

decreased intensity of the sounds and murmur caused by mitral insuciency. In more severe cases, there are more symptoms of heart failure.
Complementary tests show the following signs:
ECG: Nonspecific repolarization abnormalities or signs of concurrent pericarditis may be detected, as well as arrhythmias (extrasystoles) or conduction disorders.
Chest x-ray is usually normal during early stages. In severe cases,
it may show an enlarged heart silhouette and signs of pulmonary
congestion.
Echocardiography may disclose diuse thickening of the ventricular wall secondary to interstitial edema and changes in contractility
that are usually diuse but, occasionally, segmental. The volume of
the cavities is usually normal or slightly increased, unlike the volume
in dilated cardiomyopathy.
Cardiac magnetic resonance imaging is more sensitive and specific
than echocardiography for the detection of inflammatory processes
and interstitial edema.
Laboratory tests indicate an increase in necrosis markers, such as
troponin or CPK-MB, especially in earlier stages.
Endomyocardial biopsy should be considered the reference standard for diagnosis although this is not usually necessary.
The proposed diagnostic criteria (Table 65) include clinical suspicion
of myocarditis if one or more clinical criteria and one or more of criteria based on complementary test exist. In addition to these requirements, there needs to be absence of significant coronary heart disease, and other types of heart disease or systemic diseases that could
otherwise explain the clinical symptoms (e.g., valve disease, congenital heart disease, and hyperthyroidism). In asymptomatic patients,
two or more criteria based on additional tests must be fulfilled:
Myocarditis is usually a self-limiting disease but, in those rare cases
when it is fulminant, it progresses rapidly and death occurs in less than
two weeks. However, if the acute phase is overcome, the heart may return to normal. In subacute or chronic cases, it may evolve into chronic
dilated cardiomyopathy.
ECG/Holter/stress test. Detection of a

new disturbance in any of these tests:
atrioventricular block or bundle branch
block, repolarization abnormalities
(ST segment elevation or depression,
T-wave inversion), ventricular
arrhythmias, atrial fibrillation,
new Q waves, R wave reduction,
intraventricular conduction disorder
(nonspecific QRS widening), frequent
extrasystoles
CRITERIA BASED
ON COMPLEMENTARY II. Elevated markers of myocardial
damage
TESTS
III. Functional/structural alterations
in cardiac imaging techniques
(echocardiography/cardiac magnetic
resonance/ventriculography). New
systolic or diastolic function alterations
only explained by this condition
IV. A cardiac magnetic resonance reveals
edema and/or late gadolinium
enhancement pattern compatible
with myocarditis
Table 65. Diagnostic criteria for myocarditis
14.4. HIV-Related
Cardiomyopathy
HIV-reacted cardiomyopathy appears relatively frequently in patients
with HIV and it is generally asymptomatic. Left ventricle aectation is
more frequent and in some cases it appears to be directly caused by HIV.
In other cases, myocarditis is due to opportunistic pathogens that take
advantage of immunosuppression. Nutritional deficiencies, toxic agents
or the medication used may also promote myocardial damage.
For treatment, rest is indicated as well as specific drugs for heart failure (diuretics, ACE inhibitors and beta-blockers). In subacute or chronic
cases that do not improve with standard treatment of heart failure, immunosuppressants (corticosteroids, azathioprine or cyclosporine) can
be administered.
14.5. Bacterial and Parasitic
In fulminant cases, intensive treatment of heart failure is preferred over

immunosuppressant drugs, including balloon counterpulsation and/or
Bacterial myocarditis is rare. It generally occurs as a complication of

infective endocarditis. Producing abscesses near valve annulus mainly
Myocarditis
79
caused by S. aureus and enterococcus. In exceptional cases, hematogenous dissemination in sepsis, by almost any bacteria, may cause intramyocardial microabscesses often masked by other manifestations of
the disease.
Diphtheric myocarditis (C. diphtheriae). Cardiac involvement is
the most frequent cause of death in patients with diphtheria. It
can produce cardiomegaly with myocardial hypocontractility, as
well as arrhythmias and, typically, atrioventricular block (diphtheria toxin has particular anity for the cardiac conduction system).
Treatment consists of early administration of antitoxin and antibiotics.
Toxoplasma myocarditis (T. gondii) appears in immunocompromised adult patients (in children, it usually occurs as congenital
toxoplasmosis). In toxoplasma myocarditis, there is cardiac dilatation with heart failure, pericarditis with pericardial eusion and
rhythm and conduction alterations.
Lyme carditis (Borrelia burgdoreri) cardiac abnormalities develop
in up to 10% of cases, mainly atrioventricular block in the AV node,
condition that may require pacemaker implantation, but is usually
transient. Heart failure is exceptional.
Whipples disease (Tropheryma whippleii) can aect the heart
structures. The agent causing this disorder is located in the myocardium or coronary arteries. It is linked to macrovascular or microvascular ischemia. Myocarditis is usually mild, although there have
been reports of manifest heart failure.
Chagas disease (Trypanosoma cruzi). This disease is endemic in
Central and South America and it is a first order health problem,
as it is estimated that more than 20 million people are infected.
Immigration is a responsible factor for the increase in the number
of cases detected in our area. The protozoan Trypanosoma cruzi
is found in the digestive tract of the assassin bug that lives in
thatched roofs in rural endemic areas. At night, it comes down and
bites humans, mostly children, usually near the eyes. The bite itself
is not infectious, but contact with the feces of the bug with the sting
site or with conjunctival mucosa originates an acute infection by the
protozoan. Few patients with Chagas disease present an acute cardiac involvement (10%); however, when cardiac aectation occurs,
it can be fatal, with symptoms of acute fulminant myocarditis by
parasite migration to the myocardium and an intense local inflammatory response.
Usually, after a latency of 20 years, up to 30% of patients present clinical
symptoms because of damage of nerves and ganglia of the autonomic
system (the megaesophagus or megacolon are frecuent) and, cardiac
involvement. Myocardial damage appears to be caused by the parasite
itself and by the autoimmune response of the body that releases autoantibodies that act on the sarcoplasmic reticulum, the beta-adrenergic
receptor and other structures of the cardiac cells. Parasympathetic denervation as a result of ganglia involvement is a characteristic complication. Rhythm alterations are frequent.
Vegetative denervation usually produces sinus bradycardia and chronotropic incompetence, even in the presence of heart failure. Involvement of the conduction system with anterior hemiblock and right
bundle branch block are frequent markers of cardiac involvement.
Arrhythmia is relatively frequent, both atrial fibrillation and ventricular arrhythmia, related to physical activity in particular, and there can
be isolated extrasystoles, nonsustained or sustained tachycardia by
reentry through myocardial scars, which may lead to sudden death
(Figure 119).
80
Myocardial involvement is characterized by hypokinesia of the posterior

basal wall of the left ventricle with relative sparing of the interventricular
septum, apical aneurysm formation frequently occupied by a thrombus,
and progressive deterioration of myocardial function that mimics dilated
cardiomyopathy. Right chambers dilatation secondary to pulmonary hypertension is a usual pattern that can be detected in advanced stages. Treatment of cardiac involvement is similar to that of chronic heart failure. ACE
inhibitors, beta blockers, diuretics, and other medications are administered.
Figure 119. 12-lead EGC showing characteristic signs of heart

involvement in Chagas disease. Note the low voltage of QRS, infrahisian
conduction disease and frequent ventricular extrasystoles
Anticoagulation is eective for the prevention of embolism. The implantable defibrillator is used as in other heart conditions. Amiodarone may be
eective in preventing ventricular arrhythmias. Antiparasitic agents are
useful in acute cases, but the benefit is very limited in advanced stages of
the disease. The most eective measure is, undoubtedly, prevention and
deworming endemic areas with the use of insecticides to kill the vector.
14.6. Giant Cell Myocarditis

This is an inflammatory disease of the myocardium that may be associated with fatal heart failure and arrhythmias and, on endomyocardial
biopsy, multinucleated cells can be observed. Giant cell myocarditis
etiology is unknown and it is sometimes associated with autoimmune
diseases, such as inflammatory bowel disease, disseminated lupus erythematosus, thyrotoxicosis, or with infectious myocarditis.
Prognosis is very poor despite treatment with powerful immunosuppressants, and these are generally patients who, provided there are no
contraindications, should be assessed for cardiac transplantation.
14.7. Radiation Myocarditis

While the heart is particularly resistant to acute radiation damage, radiotherapy can occasionally induce acute pericarditis or mild transient
Card iology
systolic dysfunction. In some patients, cardiac impairment appear years

after exposure to radiation, usually in the form of myocardial fibrosis. It
is similar to restrictive cardiomyopathy as regards impaired microcirculation with occasional calcification and valvular fibrosis that produces
associated valvular functional impairment. It can also cause late acute
pericarditis or stenosis in the coronary stenosis.
14.8. Cardiac Tumors

Secondary or metastatic cardiac tumors (breast tumors in women and
lung tumors in men) are much more common than primary tumors.
Myxoma is the most common primary cardiac tumor and it is usually
located in the interatrial septum towards the left atrium (Figure 120).
From the histologic point of view, cardiac myxoma is usually benign.
Since it is a friable tumor, there is risk of fragment embolization.
In cases of myxoma, elevated levels of cytokines can produce systemic
symptoms such as fever, weight loss, Raynauds disease, increased sedimentation rate. Diastolic tumor plop is a characteristic sign detected on
auscultation.
The preferred treatment for cardiac myxoma is surgery.
Malignant primary cardiac tumors tend to be sarcomas, and prognosis
is poor.
Figure 120. (A) Apical echocardiographic image of the four chambers

revealing an atrial myxoma (Mx). (B) The same image, obtained after
injection of a contrast agent. (C) Echocardiographic image
of the same patient (subcostal view)
81

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Cardiology

A m e r i c an Ma nua l o f Ex am i na ti o n i n Me dicine (2CK)

Cardiac Auscultation ........................................................................................................ 1

Cardiac Frequency ............................................................................................................... 4

05. Cardiomyopathies ............................................................................. 27

Dilated Cardiomyopathy ........................................................................................ 28

06. Coronary Artery Disease ......................................................... 35

07. Acute Coronary Syndrome .................................................. 40

7.1. Acute Coronary Syndrome without

04. Heart Failure ............................................................................................... 17

08. Dyslipidemia .............................................................................................. 48

03. Arrhythmias .................................................................................................... 6

10. Pericardial Disease............................................................................ 53 13. Syncope ........................................................................................................... 75

11. Valvulopathies ......................................................................................... 58

Basic Concepts ...................................................................................................................... 58

12. Vascular Disease .................................................................................. 67

13.1. Differential Diagnosis.................................................................................................. 75

14. Miscellaneous .......................................................................................... 77

of the apex instead of an apical impulse is detected, constrictive

Some cardiac diseases present characteristic features when observed

1.1. Cardiac Auscultation

Posterobasal left ventricular

Lower limbs pseudohypertrophy

Inexpressive myopathic facies

Aneurysm or aortic dissection

Skin and joint hypermobility

Hemorrhagic telangiectasia in mucous

Flushing and edematous episodic

Lachrymal glands hypertrophy

Very tall. Enlarged limbs

Hypercholesterolemia Coronary artery disease

Downward dislocation of the lens

Infective endocarditis Endocarditis

The first heart sound (S1) is produced by

Roth spots in retina

A wide and fixed splitting of the second

A m e r i c an Ma nua l o f Ex am i na ti o n i n Me dicine (2CK)

ture, anemia or pregnancy, it is frequent to auscultate a mild mesosystolic

HOCM and MVP

HOCM and MVP

Aortic stenosis, HOCM, Mitral and aortic

Mitral and aortic

Aortic stenosis, HOCM,

Table 2. Maneuvers helping determine the nature of murmurs

1.3. Arterial Pulse

Figure 1. Auscultation focuses

Increase in cardiac output volume,

Hypovolemia, LV insufficiency (AMI,

Aortic insufficiency, HOCM

Two systolic peaks

Dilated cardiomyopathy in low

Two peaks: one, systolic and the

Decrease in cardiac output volume

Flat (weak) and prolonged wave

In ventricular decompensation, with S3

Pulse amplitude variation

Ventricular premature contraction,

Alternation of normal and

Cardiac tamponade, respiratory

More than 10 mmHg drop in

Table 3. Some abnormalities of arterial pulse

rounded peak (called pre-dicrotic wave), followed by a slower decline,

1.4. Jugular Venous Pressure

Central venous pressure may also be measured by estimating the height