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CARDIAC ANATOMY
EMBRYOLOGY
Early cardiogenesis
Morphologic stage
The morula and the plate will fuse to form your primitive heart. They
all came from angiogenic cell clusters. At 22 days of age, there are 2
pairs of tubes.
Divisions of primitive heart:
1. Truncus becomes aorta
2. Bulbus cordis divided into 2
3. Old ventricle
4. Atrium
Bulbus cordis becomes corus cordis and bulbus cordis; becoming
left and right ventricles. Atria will be divided into 2: the right and the
left atria, and then the sinus venosus.
Truncus forms aorta and pulmonary artery.
Bulbus right ventricle
Conus cordis becomes outflow tract for both ventricles. The right
and the left ventricle will have its own outflow tract. However the
conus in the left ventricle will be absorbed unlike in the right
ventricle, it has a long outflow tract because the conus on the right
is not absorbed.
Primitive ventricle will become left ventricle.
CARDIAC LOOPING
Straight tube will loop prior to cardiac looping.
During cardiac looping: D loop or L loop
If you have an infection on the 22 day where you do not have yet
the valves, you may have a patient with just a univentricular heart.
The earliest time that the mother has the infection, the worse is the
heart disease.
CARDIAC SEPTATION
You forming the interatrial septum. During the formation of the
interatrial septum,, initially you have a common atrium then you will
form a septum. The first one, upper one will outpouch. Septum
primum is the first to form. The hole between your endocaridal
cushion and your septum primum is your ostium primum. Primum
first to form after which, closure. Septum secundum grows to close
it fully. When they are closed fully, you are going to have a hole in
that septum coalesce to form you foramen ovale. Your foramen
ovale is important in the cardiac cycle because your fetus has a
parallel circulation.
Hole created is called foramen segundum/ostium segundum.
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NEONATAL CIRCULATION
1.
2.
3.
4.
5.
Prenatal
Medications
Exposures esp. congenital; term cyanotic delivery:
exposure to Rubella, PDA in congenital rubella syndrome;
rd
when did the mother have a problem? Infection on 3
month of gestation cardiac anatomy is already formed,
probably pulmonary artery is stenosed or there is a lodged
shunt; exposure/illness at 1 month of gestation: TGA,TOF;
th
th
7 to 9 month of gestation, most do not manifest with
cyanotic heart disease more of the infection
Signs and symptoms depends on what age patient is:
infant feeding history (sleeping during feeding), how
many ounces does the baby consume? Manifestation of
congestion is with interrupted feeding. Upon feeding there
may be diaphoresis. Pattern of breathing e.g. fast
breathing, fatigability; cyanosis e.g. 1 month when place
baby in air conditioned room (acrocyanosis)
Chest pain: 15 y/o male, comes to you with chest pain started a
year ago, even at rest, more svere pain during activity, sometimes
relieved by rest, pain scale 5/10, does not hinder normal activities,
feeling of something on the chest, radiates to left and back
sometimes, sometimes stomach hurts, Ranitidine, Omeprazole,
Mallox; mother and sibling has RHD, cousin operated for valve
replacement; Sumapen for the last 5 years but sometimes forgets to
take it (prophylaxis for RHD); having it for a year, pain with activities
= RHD
Patients with aortic regurgitation may manifest
Heart failure feeding, diaphoresis, fatigue, breathing, respiratory
distress, dyspnea, cyanosis (look at tongue)
PE
-
S1 closure of AV valves
S2 closure of pulmonic and aortic valves
A1
P2
Split deep inhalation
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Good Hx
Good PE
CXR PA view or lateral view; right side: moguls RV on
anterior; on right SVC, RA, RV; LV will not produce a
shadow on PA view because it is located posteriorly;
nd
enlargement of 2 mogul on the left think of PDA or VS
ECG demonstrate anatomic and hemodynamic system;
13 lead 15 lead 18 lead
Echocardiogram
The QRS amplitude would tell you if you have a tall R it depends on
th
the right or left leads. V1 tall R; V1 and V2 at level of 4 ICS; RV look
at V1, LV look at V5, V6 and V7. If you see a tall R in V6 you have left
ventricular enlargement
P wave = normal axis is 90
QRS complex ventricular hypertrophy; also tells us of the systolic
overload pattern if you see tall PQR in right precordial leads (V1) or
diastolic overload pattern RSR in primum ASD.
Hematologic = request as needed
Patient severely cyanotic = request for CBC to know if the patient is
Polycythemic. The normal Hct in a cyanotic patient is up to 0.6.
Above that we do phlebotomy.
3D echo
TEE (transesophageal echocardiogram) insert a probe through
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your esophagus at the level of your heart, 4 intercostal; better
done in patients for operation because they are sedated
Fetal echocardiography
Exercise testing Stress test
MRI/CT
Radionucleide study
CT angiography evaluate the structures not seen during echo, in
patients with double aortic arch, long segment coarctation of the
aorta, evaluate peripheral stenosis; insert probe in inguinal area
towards the heart and get the pressures to know if patient is
operable or not
Angiogram put a dye to see if there is stenosis, get pressures within
heart
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ETIOLOGY
Chromosomal abnormalities
Dilated Cardiomyopathy
Long QT syndrome
Get thorough family history once you have a patient with CHD;
parents should go into genetic counseling. Most of the time if you
have a patient with ASD, ask the siblings, they may have an
increased incidence of another patient/sibling with congenital heart
disease.
BASIC TOOLS IN CARDIOLOGIC EVALUATION OF INFANTS AND
CHILDREN
History
PE
CXR
15 lead ECG
2DED
CATEGORIES OF CHD:
1. Acyanotic
Obstructive lesions
Regurgitant lesions
2.
Volume overload
Pressure overload
L R Shunts
Qp:QS 2:1
Heart Failure
Aorta
Catecholamines
Pulmonary stenosis
Aortic stenosis
Coarctation of
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Valvular regurgitation
MR
TR
AI
PR
Insufficiency is the same with regurgitation (backflow) e.g. mitral
valve should close during systole for unidirectional flow of blood.
During systole, if your mitral valve is open, some of your blood will
go back to the LA.
pulmonary blood
RVH
VSD
PDA
AVSD
ASD
PAPVR
Eisenmengers Phenomenon
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CLASSIFICATION OF VSD
1. Perimembranous most common, 80%
2. Outlet 5-7%
3. Inlet 5-8%
4. Muscular 5-20%
a. central
b. apical
c. marginal
d. swiss cheese septum multiple muscular
Determinants of L R shunt
1. Size of VSD
2. Difference in resistance between pulmonary
systemic circulation
3. Difference in pressure between RV and LV
Shunt flow occurs in systole from beginning of systole to
end of systole you will hear the sound. That is why it is
called a pansystolic murmur.
Biventricular will occur late because in the long run if
there is continuous increase in pulmonary blood flow your
pulmonary artery is sclerosed so there will be thickening
producing an increase in your PVR so there will be an
increase in your PAP RV has to compensate by increasing
in size.
Laboratory Findings
CXR : PVN, cardiomegaly
ECG : LAE, LVH
ECHO : location/size of VSD
LA and LV size
Amount and direction of shunt
Estimate PA pressure
UNTREATED VSD
irreversible changes take place in the
pulmonary arterioles
= Pulmonary vascular obstructive
disease (Eisenmengers disease) late stage of VSD
VSD
and
PVR -- L R shunt
Signs/Sx: cyanosis- if patient develops
cyanosis
and
pulmonary
arterial
hypertension before they would only last
for about 2 years more after the onset of
pulmonary hypertension; but with the
outbreak of medicine there is Sildenafil
very effective in decreasing pulmonary
arterial pressure (0.25 mg/kg/day; paper
tab 100 mg divided into 1 paper tabs to
make 10 mg/paper tab); Milrnone increases
both cardiac contractility and decreases
pulmonary arterial pressure.
loud, single S2
CXR : RVH
5-10% of CHD
Determinants of L R shunt
1. length and diameter of ductus
2. Relative resistances of Ao and PA
Shunt flow occurs in systole and diastole continuous
nurnur
Laboratory Findings
CXR : PVM, cardiomegaly
ECG : LAE, LVH
ECHO : size of ductus and gradient
estimate PA pressure
UNTREATED PDA
PVOD
bidirectional shunting : differential
cyanosis increase PAP, increase PVR
Eisenmenger phenomenon, if not treated
you will develop pulmonary arterial
hypertension or Esienmenger phenomenon
( PAP, PVR and cyanosis)
PE : loud single S2
CXR : normal heart size;vdilated central and
main
PA
ECG : RVH
ACYANOTIC HEART DISEASE
PBF
PBF
LVH
RVH
LVH
RVH
PDA
PS
VSD
PS
AVSD
ASD
AS
VSD
PAPVR
COA
W/
PERIPHERAL
EISSEMENGER
Congenital MS produces LAE, you have a small MV, the blood from
the LA cannot pass through, it has increased pressure, as pressure
increases it will be reflected to the lungs = pulmonary edema, RVH in
the long run. When you develop heart faiure, the right side of the
heart enlarges and you are going to have hepatomegaly.
Aortic stenosis small aortic valve or there is an obstruction before
your aortic valve; during systole LV will contract, the blood will not
be able to go or ass through your aortic valve because it is small or is
obstructed, LV has to increase in pressure, LV enlarges.
Coarctation of the Aorta stenosis in your aorta; the most common
COA is type 1; after third branch is the first (type 1), after second
branch is second (type 2), after the first branch the third (type 3); if
ypu have a constriction, the blood from the aorta, the pressure is
reflected to the LV, has to increase in pressure, in size to push the
blood and pass through the constriction
VSD with PS the patient would have developed cyanosis unless the
patient is having right sided heart failure. Since you have increased
pressure on the left side, even if you have stenotic valve still the
blood will be pushed to the pulmonary artery. But if you have
pulmonary valve atresia, the blood from the right will go to the
aorta, no cyanosis; TOF has cyanosis because there is malposition of
your great vessels
Peripheral pulmonary stenosis the pulmonary arteries which ais
very near your lungs are stenosed
Volume overload
Pressure overload
Valvular regurgitation
MR
TR
AI
PR
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Categories of CHD
1. ACYANOTIC
Shunts
Obstructive Lesions
Regurgitant Lesions
2. CYANOTIC
CYANOTIC CHD
Blood flow
PBF + RL shunt
e.g. TOF most common
common
Blood flow
Abnormal VA connections
e.g TGA
most
Total mixing
TETRALOGY OF FALLOT
Large VSD
RVH
PHYSIOLOGY OF TOF
Normal O2 sat on the right is about 60% (60 65); same amount of
O2 sat will go to RV and then mixes with the high O2 in the left.
Patients with TOF are desaturated.
Moderate RVOTO
Near-normal Qp:Qs, sPO2 low 90s
Patient are asymptomatic
Severe RVOTO
Significant R-L shunting across the VSD
sPO2 at 70s
Newborns/infants- diagnosed when they present with
cyanosis or systolic ejection murmur; cyanosis usually
presents at 1 month of age in TOF but if you have
pulmonary atresia you will have cyanosis at birth
Mortality rates:
Clinical Manifestation
Hypercyanotic episodes (Tet Spell)
secondary to pulmonary vasospasm
pulmonary artery, more cyanosis, acidosis,
may die of hypoxemia; have to break cycle
of cyanosis and vasospasm
Characterized by severe and prolonged
decrease in arterial saturation cause by
abrupt changes in Qp:Qs (Inc R-L shunting)
Caused by sudden decrease in systemic
vascular resistance or dynamic changes in
the degree of subpulmonic obstruction
Irritability, hyperpnea, marked cyanosis,
pallor, lethargy of unconsciousness
(+)severe hypoxia secondary metabolic
acidosis hyperpnea/ inc in pulmonary
resistancemore R->L shunting brain
damage
Breaking the cycle IMPORTANT
Physical Examination
Reflect combination of PS, right ventricular
hypertension, and any R-L shunt
Infants are generally full-sized although
growth failure may occur overtime; most
infants are chubby as compared to your
shunt anomalies they are cachectic.
Cyanosis of varying degree depends on
degree of RVOTO
Accentuated RV impulse
S2 single and loud
Widened pulse pressure if with PDA,
collaterals or shunt
Murmur- harsh systolic secondary to
pulmonary stenosis
LUSB in location
crescendo-decrescendo
occasional AR murmur
Diagnostics
Parasternal Long-Axis
Associated AI is appreciated
Short-axis view
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Interventional:
1) balloon valvuloplasty
2) coil embolization of
MAPCAs
CXR
Vascularity is diminished in
proportion to the degree of
cyanosis
Hematologic
Polycythemia
CHB
Atretic TV
Dimple floor of the RA
Membrane
Classification of TVA
Type I (70%)
normally related great arteries
occurs in 70% to 80% of patients with TVA
Type II (28%)
d-transposition of the great arteries
occurs in 12% to 25% of patients
PVA IVS (pulmonary valve atresia with intact septum) you do not
have a flow from your RV to the pulmonary artery plus you have
pulmonary valve atresia; IVS means you do not have an intact
ventricular septum blood from RA to RV will go back to your RV, you
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EBSTEINS ANOMALY
CYANOTIC CHD W/PBF
TGA W/O PS
HLHS (hypoplastic left heart syndrome) you do not have left side
of the heart, you have a very small aorta, only have large pulmonary
artery
DORV W/O PS
TAPVR
TAPVR- Classification
A. Based on the anatomic site of the abnormal connection
I. Supracardiac (40-50%)
II. Cardiac (18-31%)
III.Infracardiac (13-24%)
IV.Mixed (5-10%)
GOD BLESS!
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