Clinical profile of restrictive cardiomyopathy

The characteristic hemodynamic features of restrictive cardiomyopathy (normal or reduced cardiac index, normal ventricular systolic function, and "dip and plateau" early in diastole) are traditionally associated with pathologic evidence of inflammation, infiltration and fibrosis. Prognosis is usually poor. Nine patients with restrictive hemodynamic features were recently identified in our laboratory; six were males, three were females, and ages ranged from 23-57 years (mean 47 years). Only one was asymptomatic. Chest pain, dyspnea on exertion and fatigue were the most common symptoms. Echocardiography revealed various degrees of left ventricular wall thickening, but no significant pericardial effusion, pericardial thickening or calcification. Mean left ventricular end-diastolic pressure was 25 mm Hg, cardiac index 2.8 l/min/m2 and ejection fraction 0.63. Endomyocardial and pericardial biopsies, obtained in two patients, were normal. Follow-up (mean 22 months, range 16-42 months) revealed no cardiac deaths. These findings support the hypothesis that the restrictive hemodynamic profile does not necessarily indicate the presence of a specific pathologic process in the subendocardium or myocardium and that the prognosis is not necessarily ominous. The common pathophysiologic feature for this syndrome appears to be reduced ventricular diastolic compliance, but the etiology in many cases is unclear.