Circulation, Vol 70, 165-169, Copyright © 1984 by American Heart Association
RJ Siegel, PK Shah and MC Fishbein
This report details the clinical, hemodynamic, and morphologic data from
four patients 59 to 77 years old (mean 66) with a primary restrictive
cardiomyopathy. All patients had symptoms of congestive heart failure,
jugular venous distention, and murmurs of mitral and tricuspid
regurgitation. Four patients required pacemakers, three for the brady-tachy
syndrome and one for complete heart block. Chest x-ray demonstrated
cardiomegaly in all four patients and pulmonary congestion and/or pleural
effusions in three. Echocardiographic examination documented left atrial
enlargement in all patients, along with normal left ventricular internal
dimensions. Global left ventricular systolic function was normal in all,
and left and right ventricular filling pressures were elevated and similar
in three. A dip and plateau pattern was present in the pressure tracings of
two of three patients. Unlike previous reports of restrictive
cardiomyopathy, in our four patients there was no specific morphologic
cause noted at necropsy. Pathologic evaluation demonstrated biatrial
dilatation in all patients, with thrombi present in the atrial appendages
in three. Normal ventricular cavity size and mild right ventricular
hypertrophy were present in all patients and mild-to-moderate left
ventricular hypertrophy was present in two. There were no significant
pericardial, endocardial, or valvular abnormalities and no infiltrative
myocardial disorders were present. Light and electron microscopic studies
demonstrated only interstitial fibrosis of the myocardium. A restrictive
hemodynamic profile may be observed in the absence of specific infiltrative
disorders and affected patients may exhibit a prolonged clinical course of
4 to 14 years (mean 9). However, in these patients congestive heart failure
responded poorly to medical therapy or surgical correction of valvular
regurgitation, which is common in this disorder.
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