Cardiovascular System:

Rheumatic Heart Diseases,

Hypertension
& Cardiomyopathy
DR A. O. OLUWASOLA.
 Rheumatic heart disease
• A sequelae of rheumatic fever, can be
acute or chronic.
• Rheumatic fever is an acute
immunologically mediated multi system
Inflammatory disease.
• It occurs 10 days to 6 weeks after an
episode of groupA (B-hemolytic)
streptococcal (pharyngitis) and often
involves the heart.
• Diagnosed by Jones Criteria:
• Either two of the major manifestations or
• One major and two minor
manifestations.
 Clinical features ctd.
• Evidence of preceding group A
• Streptococcal infection (Antistreptolysin-O titre)
• Major manifestations include:
• Migratory poly-arthritis of the large joints;
• Carditis; Subcutaneous nodules;
• Erythema marginatum of the skin and; sydenham
chorea- a neurologic disorder with involuntary
purposeless rapid movements.
• Minor manifestations
• Non specific sign and symptoms which include
fever, Arthralgia or elevated acute phase reactants.
 Clinical features ctd.
• Acute rheumatic fever appear most often in children
between the ages 5 and 15 years,
• While about 20% of 1st attacks occur in middle to later
life.
• Overall the prognosis for the primary attack is
generally good and
• Only 1% of patients die from rheumatic fever.
• Increased vulnerability to reactivation of the disease
with subsequent pharyngeal infections.
• Carditis is likely to worsen with each recurrence and
damage is cumulative.
 Pathogenesis
• Acute rheumatic fever is believed to be a
hypersensitivity reaction,
• But the exact pathogenesis remains uncertain.
• Autoimmune mech. has been proposed
• Antibodies directed against the M proteins of certain
strains of streptococci cross-react with tissue
glycoproteins in the heart, joints and other tissues.
• The chronic sequelae result from progressive fibrosis of
both healing of the acute inflammatory lesion,
• And the turbulence induced by ongoing valvular
deformities.
 Morphology
• Acute rheumatic heart disease.
• Typically occurs as a pancarditis.
• Diffuse inflammation and aschoff bodies may
be found in any of the three layers of the heart.
• Aschoff bodies are foci of fibrinoid
degeneration surrounded by lymphocytes,
• Occasional plasma cells and plump
macrophages called anitschkow cells
• pathognomonic for rherumatic fever or
caterpillar cells
• + aschoff giant cells - multinucleated cells.
Aschoff body in a patient with acute
rheumatic carditis
• Pericardium- fibrinous pericardial
exudate
• Bread and butter pericarditis
• Generally resolves without sequelae.
• Myocardum –
• Shows scattered aschoff bodies within
the interstitial connective tissue
• often perivascular
 Endocardium
• Can be involved along with cardiac
valves (usually left sided) in form of
fibrinoid necrosis within the cusps and
• Small (1-2mm) irregular vegetations –
verrucae – along the lines of closure of
the valves.
• Mac callum patch – irregular
thickenings due to subendocardial lesions
Acute and chronic rheumatic
heart disease
• Clinical features of acute carditis include
pericardial friction rubs,
• Weak heart sounds, tachycardia,
arrhythmias,
• Cardiac dilatation which may cause
functional mitral valve insufficiency or
even heart failure.
 Chronic rheumatic heart
disease
• Characterized by organization of the acute
inflammation and subsequent deforming
fibrosis and neo-vascularisation.
• 99% of cases of mitral stenosis is due to RHD.
Mitral value alone-65 to 70% of the cases.
• Mitral and aortic valve -25% .
• -Leaflet thickening and fusion of the tendinous
cords.
• Commissural fibrosis + calcification → fish or
button hole stenosis.
Chronic Rheumatic Heart Disease
• Severe mitral stenosis progresses to left atrial
hypertrophy and dilatation,
• Mural thrombosis,
• Pulmonary congestion,
• Pulmonary vascular sclerosis and then right
ventricular hypertrophy.
• The left ventricle is normal is isolated pure
mitral stenosis.
• Other complications of chronic RHD include
heart failure,
• Arrhythmias particularly AF in case of M.S,
• Thrombo embolic complications and infective
endocarditis.
• The long term prognosis is highly variable
• Rx- Surgical replacement of diseased valves
with prosthetic device .
The pathogenetic sequence and
key morphologic features of
acute rheumatic heart disease.
 Background
• HYPERTENSION – Elevated blood pressure.
• A sustained diastolic pressure greater than 90 mmHg
or
• A sustained systolic pressure in excess of 140 mmHg
• About 1I.2% of Nigs. aged 15 years and above- 1997
national survey 160/95 mmHg
• 20% of adult popn- Western industrialised countries
• Prevalence increases with age,
• Black individuals are affected by hypertension about
twice as often as whites and
• Blacks seem more vulnerable to its complications.
 Classification
• I. 10 or 20
• II. Systemic or Pulmonary
 Primary & Systemic. About 90 to 95% -
idiopathic /primary (essential HT).
 Remaining 5-10% most are secondary to
renal disease (renovascular HT).
• Categories: mild 95-104, mod 105-114,
severe >115.
 Mechanisms of Hypertension
• The BP level in any individual is a complex trait
• Determined by the interaction of multiple genetic,
environmental and demographic factors, hence,
• Multiple mechanism play a role in hypertension

• These mechanisms constitute aberrations of the normal

physiologic regulation of blood pressure.
• The magnitude of arterial pressure depends on two
fundamental hemodynamic variables: cardiac output
and total peripheral resistance.
• The kidney by influencing both peripheral resistance
and sodium homeostasis, plays an important role in
blood pressure regulation.
The critical roles of cardiac output
and peripheral resistance in blood
pressure regulation.
 Mechanisms of Essential
Hypertension
• Interaction of genetic and environmental
factors
• Genetic factors e.g. Liddle syndrome,
17αhydroxylase deficiency e.t.c.
• Environmental factors e.g.
• Stress,
• Obesity,
• Smoking,
• Physical inactivity
• Heavy consumption of salt
Hypothetical scheme for the
pathogenesis of essential
hypertension
 Causes of Secondary Hypertension
Renal
Acute glomerulonephritis
Chronic renal disease
Polycystic disease
Renal artery stenosis
Renal artery fibromuscular dysplasia
Renal vasculitis
Renin-producing tumors
Endocrine
Adrenocortical hyperfunction
(Cushing syndrome, primary
aldosteronism, congenital adrenal
hyperplasia)
Exogenous hormones (glucocorticoids,
estrogen [including pregnancy-
induced and oral contraceptives],
sympathomimetics
Pheochromocytoma
Acromegaly
Hypothyroidism (myxedema)
Hyperthyroidism (thyrotoxicosis)
Pregnancy-induced
 Causes of Secondary
Hypertension ctd
Cardiovascular Neurologic

Coarctation of aorta
Psychogenic

Polyarteritis nodosa (or other

vasculitis) Increased intracranial pressure
Increased intravascular volume
Sleep apnea
Increased cardiac output

Acute stress, including surgery

Rigidity of the aorta
 Accelerated/malignant HT
• –Seen in about 5% of hypertensives – rapidly rising
BP,
• Usually super imposed on preexisting HT.
• The clinical syndrome is characterized by:
• Severe hypertension (i.e., systolic pressure over 200
mm Hg, diastolic pressure over 120 mm Hg)
• If untreated leads to death in 1 or 2 years.
• Causes renal failure and
• Retinopathy: Retinal haemorrhages and exudates +
papilloedema,
• Brain – Charcot Bouchard microaneurysms,
Intraparenchymal haemorrhages, hypertensive
encephalopathy.
 Vascular pathology
• Affects both small & large vessels:
• Two main forms of small blood vessels diseases:
3. Hyaline arteriolosclerosis:
• Encountered generally in elderly and diabetics but
more generalized and more severe in hypertensives.
• Vascular lesions consists of homogenous pink, hyaline
thickening of the walls of arterioles and with
narrowing of the lumen.
• Presumably the chronic hemodynamic stress of HT
accentuates endothelial injury → leakage of plasma
proteins and hyaline deposition with narrowing and
• Consequent ischaemia and organ shrinkage as in
benign nephrosclerosis.
Hyaline arteriolosclerosis
2-Hyperplastic Arteriosclerosis
• Related to more acute and severe elevations of BP,
• Therefore xristic of malignant HT (diastolic press >
110 mmHg)
• Manifests as onion –skin concentric laminated
thickening of the walls of arterioles with progressive
narrowing of lumina
• Often accompanied by deposits of fibrinoid material
(fibrinoid necrosis) and
• Acute necrosis of the vessels walls – necrotizing
arterolitis.
• All tissues throughout the body may be affected but
the favoured site is the kidney.
Hyperplastic arteriolosclerosis
(onionskinning)
Atherosclerosis
 HEART
• Hypertensive heart disease:
• Systemic= left sided; pulmonary= right- sided
• Systemic: diagnostic criteria –
• (i) LVH – usually concentric in the absence of any
cardiovascular pathology that might have induced it
and
• (ii) A history or pathologic evidence of hypertension.
• This hypertrophy is an adaptive response to pressure
over load which can lead to myocardial dysfunction,
cardiac dilation, congestive heart failure and sudden
death.
Normal Heart
Hypertensive Heart Disease
 Morphology of Heart
• Circumferential hypertrophy without dilatation of
the ventricle
• Increase LV wall thickness
• Cardiomegally with increased hrt wt. → impaired
diastolic filling →
• Left atrial enlargement + AF,
• Microscopically: ↑myocyte diameter,
• later irregular with interstitial fibrosis. No
hyperplasia.
• Other possible complications include:
• IHD from the potentiating effect of HT on coronary
artherosclerosis, Cong.hrt. failure and sudden
cardiac death as above.
 Pulmonary HT
• Pulmonary vasculature
• Primary pulmonary hypertension
• Secondary causes:
• Chronic obstructive pulm. disease,
• Pneumoconiosis;
• Recurrent pulm. thrombo-embolism;
• Marked obesity and
• Chronic altitude sickness.

• It can be acute or chronic:

• Acute-massive pulm. thromboembolism → marked
Rt vent dilation without hypertrophy;
• Chronic – right vent. Hypertrophy, dilatation and
potentially failure (Cor pulmonale) + pulmonary
vascular sclerosis
Pulmonary Hypertension
 Renal Changes
• Benign Nephrosclerosis: Sclerosis of renal arterioles and
small arteries with resultant parenchymal ischaemia.
• Morphology – Hyaline arteriolosclerosis & fibroelastic
hyperplasia.
• (N) or shrunken kidneys + fine granularity.
• Malignant nephrosclerosis – occurs in 1-5% of
hypertensives. Morphology –
• “flea bitten” appearance.
• Fibrinoid necrosis of arterioles;
• Hyperplastic arteriolitis
• Necrotizing glomerulitis
• BP usually >130 mmHg diastolic →
Benign Nephrosclerosis.
 Malignant
Nephrosclerosis
Fibrinoid Necrosis
 BRAIN
• The most important effects of
hypertension on the brain include:
2. Massive hypertensive intracerebral
hemorrhage,- +berry aneurysms
3. Lacunar infarcts and slit hemorrhages,
4. Hypertensive encephalopathy.
Atherosclerosis and diabetes are
frequently associated diseases
Massive hypertensive
hemorrhage
 BRAIN-2
• The cerebral vessels develop arteriolar
sclerosis and may become occluded; -
TIA
• Ischaemic strokes 75-80%; hmgic-15%;
SAH-5%; 5%
• Development of single or multiple, small,
cavitary infarcts-lacunes, or lacunar
state (état lacunaire)
• Lacunes can either be clinically silent or
cause severe neurologic impairment.
• Widening of the perivascular spaces
around affected vessels - (état criblé).
Lacunar infarcts in the
caudate and putamen.
 BRAIN-3
• Slit Hemorrhages
• Rupture of the small-caliber penetrating vessels and
the development of small hemorrhages. Which
resorbs, slitlike cavity (slit hemorrhage) surrounded
by brownish discoloration;
• Hypertensive Encephalopathy
• Clinicopathologic syndrome arising in a hypertensive
patient characterized by diffuse cerebral dysfunction,
including headaches, confusion, vomiting, and
convulsions, sometimes leading to coma.
• Edematous brain with or without transtentorial or
tonsillar herniation. Petechiae and fibrinoid necrosis
of arterioles in the gray and white matter may be
seen microscopically.
 Hypertensive
Enchephalopathy
 BRAIN-4
• Vascular (multi-infarct) dementia –
• Multiple, bilateral, gray matter (cortex, thalamus, basal
ganglia) and white matter (centrum semiovale) infarcts
caused by multifocal vascular disease, consisting
largely of
• (1) cerebral atherosclerosis,
• (2) vessel thrombosis or embolization from carotid
vessels or from the heart, or
• (3) cerebral arteriolar sclerosis from chronic
hypertension.
• Binswanger disease
• Pattern of injury preferentially involves large areas of
the subcortical white matter with myelin and axon loss,
Multi infarct dementia
 EYE
• RETINAL VASCULAR DISEASE
• A- ‘Non Malignant’ Hypertensive retinopathy
1. Thickened arteriolar wall -generalized arteriolar
narrowing
2. Copper & Silver wiring. Vessels may appear narrowed,
and the color of the blood column may change from
bright red to copper and to silver depending on the
degree of vascular wall thickness

3. A – V nipping Retinal arterioles and veins share a

common adventitial sheath.
Therefore, in pronounced retinal arteriolosclerosis, the
arteriole may compress the vein at points where both
vessels cross
4. Venous stasis distal to arteriolar-venous crossing may
precipitate occlusions of the retinal vein branches.
 EYE-2
• B Malignant Hypertensive Retinopathy,
• Vessels in the retina and choroid may be
damaged. –Hmges, hard exudates
• + Optic disc oedema
• Elschnig's spots -Damage to choroidal vessels
may produce focal choroidal infarcts,
• Retinal detachment -Damage to the
choriocapillaris, the internal layer of the
choroidal vasculature, may, in turn, damage the
overlying retinal pigment epithelium and permit
exudate to accumulate in the potential space
between the neurosensory retina and the retinal
pigment epithelium.
Retinal Detachment
 EYE-3
• Occlusion of retinal arterioles may produce
infarcts of the nerve fiber layer of the retina.
• “Cotton-wool spots".
• Accumulation of mitochondria at the swollen ends
of damaged axons creates the histologic illusion of
cells (cytoid bodies).
• Collections of cytoid bodies populate the nerve
fiber layer infarct, seen ophthalmoscopically as
"cotton-wool spots".
Nerve fiber layer
infarct
 CARDIOMYOPATHY,
• Heart disease resulting from a primary
abnormality in the myocardium.
• There are main 3 recognized types:
• Dilated, hypertrophic and restrictive based
on clinical functional and pathologic
considerations.
• Arrythmogenic Right ventricular
Cardiomyopathy.
• Dilated (DCM) – Most common; xrised by
progressive cardiac hypertrophy, dilatation
and contractile (systolic) dysfunction.
The three distinctive and
predominant forms of
cardiomyopathies.
 Pathways of dilated and
hypertrophic cardiomyopathy
 Causes of Dilated
Cardiomyopathy
• Idiopathic,
• myocarditis (viral),
• Alcohol abuse,
• Doxorubicin,
• Peripartum,
• Genetic 30-40%–(dystrophin gene)
• Morphology-
• Heart is usually heavy and enlarged and flabby
with dilation of all chambers,
•
• Mural thrombi are common + thrombo-
embolism
• + functional regurgitation
• microscopy – non specific changes
hypertrophic fibres enlarged nuclei and
stretched/thin:
• Interstitial fibrosis;
• Commoner between ages 20-60 presenting with
slowly progressive congestive hrt failure,
• Death occurs within 2years usually
attributable to hrt failure or arrhythmia.
Dilated cardiomyopathy
 Hypertrophic HCM/Idiopathic
hypertrophic sub-aortic stenosis
• -Xrised by myocardial hypertrophy,
• Abnormal diastolic filling and in about
one third of cases intermittent left
ventricular out flow obstruction.
• The heart is heavy,
• Muscular and hypercontracting.
Hypertrophic Cardiomyopathy
Myofibre Disarray
 Morphology
• - There is disproportionate thickening of the
ventricular septum as compared with the free
wall of the L.V.
• Asymmetric septal hypertrophy;
• (1/3 = symmetric)
• Micro:
• Hypertrophic fibers;
• Myofibre disarray and interstitial fibrosis.
 Pathogenesis
• : 5% of cases are familial.
• AD inheritance
• - Mostly any of 4 genes coding myocardial
contratile elements may be affected,
• B-myosin heavy chain most frequently
affected,
• Others are troponin T,
• Tropomyosin,
• Myosin binding protein C.
• Clinically – there is reduced stroke volume,
• However major problems are atrial
fibrillation,
• Mural thrombosis + embolization
infective endocarditis on mitral valve,
• Intractable cardiac failure and
ventricular arrhythmias and sudden
death.
 Restrictive cardiomyopathy
• – A disorder xrized by a primary decrease in
ventricular filling during diastole;
• It can be idiopathic or associated with distinct
disease that affects the myocardium principally
radiation fibrosis,
• Amyloidosis, candidiasis, metastatic tumour or
metabolic disorders.
• Morphology – in idiopathic – bilaterial,
• Dilatation common with patchy or diffuse,
• Fibrosis and normal or slightly enlarged
ventricle.
 Endomyocardial fibrosis
• – A disease of children and young adults in
African and other tropical areas,
• Xrised by fibrosis of the ventricular
endocardium and sub endocardium that
extends from the apex toward and often
involving the tricuspid mostly,& the mitral
valve.
• It → reduced ventricular compliance and
diastolic filling + ventricular mural thrombi.
• Cause is unknown.
 Epidemiology
• – Affects people of lower socio-economic
group.
• Disease has been linked to filariasis,
• Eosinophil leucocytosis,
• Consumption of plantain and
immunological factors.