Cardiac ultrastructure in primary restrictive cardiomyopathy.

Abstract:

:A five-year clinical follow-up and the results of myocardial biopsies are described in a patient with primary restrictive cardiomyopathy. Histologic examination of a right ventricular endomyocardial biopsy taken early in the course of the illness was not contributory. Histologic examination of a left ventricular endomyocardial biopsy five years later showed hypertrophy and disarray of myocytes, thickening of the endocardium, and interstitial fibrosis. Connective tissue was compact and regularly oriented in the endocardium, but tangled and irregularly oriented in the interstitium. It is concluded that the irregular network of collagen fibrils and elastic fibers limits diastolic relaxation and prevents ventricular dilatation; that the coexisting hypertrophy results from an attempt to maintain normal pump function; and that the myocyte disarray is a consequence of abnormal mechanical forces generated under conditions of severe fibrosis.

journal_name

Chest

journal_title

Chest

authors

Arbustini E,Buonanno C,Trevi G,Pennelli N,Ferrans VJ,Thiene G

doi

10.1378/chest.84.2.236

subject

Has Abstract

pub_date

1983-08-01 00:00:00

pages

236-8

issue

2

eissn

0012-3692

issn

1931-3543

pii

S0012-3692(15)33508-X

journal_volume

84

pub_type

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