CARDIOMYOPATHIES

DR SUVARNA NALAPAT

CLASSIFICATION

Normal wt of adult heart 350 gm Physiological-exercise Cardiomyopathy-dysfunctionPrimary ,secondary Both have 3 functional states 1 hypertrophic-hyperdynamic 2 Dilated-congested 3 Restrictive-constrictive

CMP

specific

indirect

Dilated(systolic Infective Ihd,valvular,hyp disorder) myocarditis,hem ertensive,congen achromatosis, ital anemia,alcohol, Hypertrophic(di Fredrich Hypertensive,ao astolic) ataxia,glycogen rtic stenosis storage,infant of diabetic mother Restrictive(diast Amyloid,pancar Pericardial

Cardiomyopathies

Dilated (Congestive) . globoid shape-because all the chambers are dilated. flabby, and the myocardium poorly contractile..(cardiomegaly, recurrent progressive failure,signs of systemic/pulmonary embolism )

there is also hypertrophy. The most common cause is chronic alcoholism, though some may be the end-stage of remote viral myocarditis

Dilated cardiomyopathy

dilated left ventricle typical of a dilated, or congestive, cardiomyopathy. Many of these have no known etiology (so-called "idiopathic dilated cardiomyopathy") while others may be associated with chronic alcoholism. The heart is enlarged and flabby .mural thrombi in ventricle may be seen.

Dilated CMP

Microscopically, the heart in cardiomyopathy demonstrates hypertrophy of myocardial fibers (which also have prominent dark nuclei) along with interstitial fibrosis.

DILATED CARDIOMYOPATHY

Hypertrophic (left atrium dilated)50-60% familial. Micro-disarray of myocardial fibres,thickwalled intramural coronary artery.point mutations in betamyosin heavy chain gene,and troponin T gene.

The most common form, idiopathic hypertrophic subaortic stenosis (IHSS) results from asymmetric interventricular septal hypertrophy, resulting in left ventricular outflow obstruction(mirrorimage fibrous plaque)

HYPERTROPHIC

left ventricular hypertrophy, with asymmetric bulging of a very large interventricular septum into the left ventricular chamber. familial, Both children and adults can be affected, and sudden death can occur. Seen here is the explanted heart.

Hypertrophic CMP

The myocardium demonstrates "myofiber disarray" with hypertrophic cardiomyopathy, typically in the septum. Seen here at the left with H&E stain and at the right with trichrome stain are sections demonstrating these irregular myofibers with surrounding collagen. Such abnormal areas predispose to arrhythmias.

Restrictive CMP

Restrictive .

The myocardium is infiltrated with a material that results in impaired ventricular filling. The most common causes are amyloidosis and hemochromatosis

RESTRICTIVE(HEMACHRO MATOSIS,AMYLOIDOSIS)

Congo red with flourescent microscopy

Endocardial fibroelastosis

Infancy,childhoodmechanical,inflammatory,familial causes Clinical exclusion diagnosis Gross-left sided rapid increase in sizeglobular.opaque porcelain surface ,diffuse outflowtract thickening.Flat trabecule carneae cordis,thick short corde tendinae,fibrotic papillary muscles. Micro-hyperplasia of endocardium ,prominent collagen,elastic fibres

Endomyocardial fibrosis

Inflamed fibrous reaction-ventricular endocardium Loeffler-endocarditis parietalis fibroplastica Fibrosis,eosinophils in heart(tropical davies endocardial fibrosis) Parasitic,hematologic malignancy(lymphoma)carcinoma,hypersensitivit y disorder,granulomatous,vasculitis. Hypereosinophilic syndrome-restrictive cardiomyopathy

Hypertensive heart disease

This left ventricle is very thickened (slightly over 2 cm in thickness), but the rest of the heart is not greatly enlarged. This is typical for hypertensive heart disease. The hypertension creates a greater pressure load on the heart to induce the hypertrophy.

hypertensive

The left ventricle is markedly thickened in this patient with severe hypertension that was untreated for many years. The myocardial fibers have undergone hypertrophy as a consequence of the increased pressure load. The result is similar to a hypertrophic cardiomyopathy.

ISCHEMIC CARDIOMYOPATHY

scattered microscopic foci of myocardial fiber loss and interstitial fibrosis. The end result is a gross and microscopic appearance similar to a cardiomyopathy. There is significant coronary artery narrowing(absent in true CMP), but no sudden severe ischemic event occurs, rather a slower loss of fibers over the years..

secondary

Cor pulmonale Systemic hypertension Toxic injury Radiation injury Metabolicbrown atrophy,degenerations,amyloid,storage diseases,hemachromatosis,anemia,hyperthyroidis m,carcinoid heart,nutritional deficiencies