Abstract
A 16 year old boy has been followed up since 1952. Though he now experiences slight exertional dyspnea, his cardiac status has not changed during this 14 year period. The clinical and catheterization data indicate that this patient represents a case of idiopathic hypertrophic cardiomyopathy that involves the septum and has produced narrowing of the apex of the right ventricle. Marked blood oxygen desaturation and elevated pressures were found at the right ventricular apex and are believed to be due to systolic obliteration and isolation of the apex by muscular contraction. No obstruction to pulmonary flow was found, and there was no gradient across the left ventricular outflow tract at rest. Our findings are similar to some observations made by others in patients with idiopathic hypertrophic cardiomyopathy, and different from reported cases of double-chambered right ventricle. They indicate that idiopathic hypertrophic cardiomyopathy represents a wide spectrum of manifestations and may involve predominantly the right ventricle.
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