Circulation, Vol 55, 489-496, Copyright © 1977 by American Heart Association
BJ Maron, CE Clark, WL Henry, T Fukuda, JE Edwards, EC Mathews Jr, DR Redwood and SE Epstein
Echocardiographic and necropsy studies were performed in 304 patients with
various cardiac diseases. The overall prevalence of disproportionate
ventricular septal thickening (septal to free wall ratio greater than or
equal to 1.3) was 10%. However, it was related to the type of cardiac
lesion. Prevalence was high (greater than 20%) in pulmonary stenosis or
primary pulmonary hypertension, lower (less than 15%) in Eisenmenger
syndrome or aortic or mitral valvular disease and was not present in atrial
or ventricular septal defect. In right ventricular overload, prevalence of
disproportionate septal thickening correlated with increasing ventricular
systolic pressure. None of 16 patients with disproportionate septal
thickening studied at necropsy showed marked disorientation of cardiac
muscle cells in the ventricular septum, characteristic of genetically
transmitted asymmetric septal hypertrophy (ASH). Furthermore,
disproportionate septal thickening was demonstrated by echocardiography in
only one of 59 first degree relatives of patients with disproportionate
septal thickening and associated cardiac diseases. Thus, disproportionate
ventricular septal thickening associated with other cardiac diseases
usually is due to secondary hypertrophy and is not a manifestation of
genetically transmitted ASH.
ARTICLES
Prevalence and characteristics of disproportionate ventricular septal thickening in patients with acquired or congenital heart diseases: echocardiographic and morphologic findings
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