The Cardiovascular Aspects of Marfan's Syndrome: A Heritable Disorder of Connective Tissue

Abstract

Clinically, Marfan's disease behaves as an abiotrophy of some connective tissue element. Cardiovascular manifestations result from defective aortic media, defective valve cusps, interatrial communication, and pectus excavatum. The defect of the aortic media manifests itself by dissecting aneurysm, diffuse aneurysm of the ascending aorta or a combination. Subacute bacterial endocarditis in a patient with Marfan's disease is described. Interatrial septal defect is less frequent than previously believed. Cardiac symptoms in severe pectus excavatum must be evaluated in light of possible Marfan's disease. Fifty families in which at least one bona fide instance of Marfan's disease has occurred were collected.