Aortic dystrophy: a cause of hypertrophic cardiomyopathy of obscure origin

Abstract

Cardiac hypertrophy of obscure origin, with or without features of congestive heart failure, is a relatively common finding at autopsy. A primary abnormality of cardiac muscle has usually been considered the basis of this condition. This paper proposes that the basic pathologic process does not affect the myocardium, but rather the ascending aorta. This "aortic dystrophy" is characterized grossly by diffuse ectasia of the ascending aorta, and microscopically by typical histologic features. The hypertrophy of the left heart appears to be work hypertrophy secondary to increased volume of the ascending aorta and reduced elastic recoil of the aortic wall following ventricular systole. The disorder in "idopathic" cardiac hypertrophy with aortic dystrophy is heritable (mendelian dominant), also affects the skeleton, and can be demonstrated in relatives. Dissecting aneurysm of the aorta is an alternative manifestation of aortic dystrophy, and may occur in families or in patients with "idiopathic" cardiac hypertrophy.