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

TLDR

Marfan’s syndrome is a heritable connective‑tissue disorder that predisposes patients to cardiovascular complications such as aortic media defects, valve cusp abnormalities, interatrial septal defects, and pectus excavatum, which can manifest as dissecting or diffuse ascending aortic aneurysms. The study compiled data from fifty families each containing at least one confirmed case of Marfan’s syndrome. The review reports a case of subacute bacterial endocarditis in a Marfan patient and notes that interatrial septal defects are less common than previously thought.

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.