Internal Medicine

0521779407-16 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:18

Other Cardiomyopathies 1113

➣Chest X-ray: enlarged heart, pulmonary congestion (dilated);

mild cardiomegaly (hypertrophic); normal to mild cardiomegaly

(restrictive)

➣Echocardiogram: LV dilation and reduced function (dilated);

LVH, asymmetric septal hypertrophy, apical hypertrophy, sys-

tolic anterior motion of mitral valve leaflet, diastolic dysfunc-

tion, possible dynamic outflow tract obstruction (hypertrophic);

small or normal LV size, in amyloid- scintillating appearance of

myocardium (restrictive)

➣Cardiac Catheterization: LV dilation and dysfunction, high fill-

ing pressures, low cardiac output (dilated); hypercontractile LV,

dynamic outflow tract obstruction (hypertrophic); normal or

mildly reduced LV function, high diastolic pressures, square root

sign (restrictive)

■Other Tests as Appropriate

➣Biopsy (fat pad, rectal. gingival, etc) with immunohistochemical

staining for amyloidosis

➣Myocardial biopsy

➣Other tests for specific underlying etiologies (i.e. serum iron and

ferritin for hemochromatosis)

differential diagnosis

■Dilated cardiomyopathy: idiopathic, alcoholic, myocarditides,

familial, post-partum, cobalt; also arrhythmogenic right ventricu-

lar hypertrophy

■Hypertrophic: Hereditary (some forms autosomal dominant with

incomplete penetrance)

■Restrictive: Amyloidosis, endomyocardial fibrosis, inherited disor-

ders (Fabry disease, Gaucher disease, hemochromatosis, glycogen

storage diseases), sarcoidosis

management

What to Do First

■Assess underlying etiology and distinguish type of cardiomyopathy

General Measures

■Remove possible toxins and toxic exposures (i.e. cease cocaine, alco-

hol)

specific therapy

■Dilated cardiomyopathy: as per CHF