VALVULAR HEART DISEASE

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STENOSIS
Failure

of a heart valve to open completely, thereby impeding forward flow

REGURGITATION
Failure

of a heart valve to close completely, thereby allowing reversed flow

Most Common causes of Major functional valvular lesions:

Mitral

stenosis Rheumatic heart disease Mitral insufficiency Myxomatous degeneration of the mitral valve Aortic stenosis Calcification Aortic insufficiency Dilatation of the ascending aorta, related to HPN and aging

CALCIFIC AORTIC STENOSIS

Congenital

or acquired Acquired aortic stenosis:

Wear and tear of congenitally bicuspid or unicuspid valves, or anatomically normal valves Calcification of Normal aortic valves in aged individuals

 Hallmark:

heaped up calcified masses within aortic cusps Ultimately protrude through outflow surfaces into the sinus of valsalva, preventing opening of the cusps

Normal aortic valve

Stenotic aortic valve

Calcified Aortic Valve

Congenital Bicuspid aortic valve with calcification

MYXOMATOUS DEGENERATION OF THE MITRAL VALVE

One

or both mitral valve leaflets are enlarged, hooded, redundant or floppy

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Prolapse

or ballon back into the left atrium during systole

 Chordae

tendinae:

Frequently involved Elongated, thinned and occassionally, ruptured

 Annular

dilatation: characteristic (rare in other diseases causing mitral insifficiency)

Microscopic
Fibrous

layer--- attenuated Spongy layer---thickened, with deposition of myxomatous material

Possible Complications
Infective

endocarditid Mitral insufficiency requiring surgery Stroke or other systemic infarct Arrhythmias

RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

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MORPHOLOGY
Acute

RF

Widely disseminated but focal inflammatory lesions found at various sites

 Aschoff

Bodies

Foci of fibrinous degeneration surrounded by lymphocytes Plasma cells Anitschkow cells Aschoff giant cells

CHRONIC RHEUMATIC HEARTT DISEASE

Organization

of the acute inflammation with subsequent deforming fibrosis Valvular leaflets become thickened and retracted, causing permanent deformity

 Cardinal

changes:

Leaflet thickening Commisural fusion Shortening, thickening and fusion of tendinous cords Fibrinous bridging across valvular commisures ( Fish mouth/ button hole deformity)

 Mitral

valve alone 65 to 70% Mitral and Aortic 25%

MYOCARDIAL DISEASES
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CARDIOMYOPATHY

Heart

disease resulting from a primary abnormality of the myocardium

PATTERNS:
Dilated

CMP Hypertrophic CMP Restrictive CMP

DILATED CMP
Progressive

cardiac hypertrophy and

dilatation Congestive CMP

Can result from:

Myocarditis Alcohol

abuse or other toxicity Pregnancy Genetic influence Idiopathic

 Heart

is heavy, large and flabby Dilatation of all chambers Wall: Normal, thick, thin Primary valvular alterations are absent Functional regurgitation

Histology

Non-specific Does

not reflect a particular causative agent

 Hypertrophy

of cardiac muscles, with enlarged nuclei Attenuated or stretched out in some areas Interstitial and endocardial fibrosis

HYPERTROPHIC CMP

Idiopathic

hypertrophic subaortic stenosis Hypertrophic obstructive CMP

Characteristics

Myocardial

hypertrophy Abnormal diastolic filling Left ventricular outflow obstruction

 Heart

Heavy, muscular, hypercontracting DIASTOLIC disorder

 Essential

Feature:

Massive myocardial hypertrophy without dilatation Disproportionate thickening of the LV septum as compared with the free wall of the left ventricle (ratio > 1.3)

 Assymetric

septal hypertrophy Banana-like configuration of the ventricular septum

 Histologic

features:

Extensive myocyte hypertrophy Haphazard disarray of bundles of myocytes and contractile elements of sarcomeres within cells (myofiber disarray) Interstitial and replacement fibrosis

RESTRICTIVE CMP
Decrease

in ventricular compliance

 Idiopathic Distinct

Diseases that affect the myocardium:

Radiation fibrosis Amyloidosis Sarcoidosis Metastatic tumor Inborn errors of metabolism

MORPHOLOGY
Ventricles

normal or enlarged Cavities not dilated Myocardium is firm Biatrial dilatation commonly observed