Circulation, Vol 53, 9-19, Copyright © 1976 by American Heart Association
BJ Maron, WL Henry, CE Clark, DR Redwood, WC Roberts and SE Epstein
Although considerable information is available concerning the clinical
features and natural history of asymmetric septal hypertrophy (ASH) in
adults, little is known of this disease in children. The clinical
characteristics and course of 46 children with ASH, who were evaluated at
the National Heart and Lung Institute, have been analyzed. Twenty- four
children had obstruction to ventricular outflow; 22 children had no
obstruction to ventricular outflow, including 11 patients without overt
manifestations of cardiac disease other than echocardiographic evidence of
ASH. Thirty-five of the 46 children have been followed for one to 16 years
(average 7.4 years). These latter children represent that subgroup of
patients with ASH referred to the National Heart and Lung Institute and
diagnosed prior to the general availability of echocardiography. The
clinical course of these patients was variable. Fourteen (40%) of the 35
patients improved or remained stable, including four patients who received
propranolol. Ten (29%) of the 35 patients deteriorated clinically and 11
(31%) of the 35 patients died suddenly (4% mortality per year). Two of the
patients who died suddenly had previously undergone operation (six and 13
years previously) with resultant abolition of the outflow gradient; four
others were taking propranolol. Neither symptomatology,
electrocardiographic abnormalities, heart size, left ventricular ejection
or upstroke time, magnitude of outflow gradient, or left ventricular
end-diastolic pressure proved predictive of sudden death. Excluding
patients who had previous operation, eight (40%) of 20 patients with
obstruction who were followed long term and one (9%) of 11 patients without
outflow obstruction died suddenly. Thus, the clinical and hemodynamic
spectrum of ASH in childhood is broad. However, deterioration in clinical
condition or sudden death has been relatively common in children with overt
signs of cardiac disease.
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Asymetric septal hypertrophy in childhood
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