Heritable disorders predisposing to aortic dissection.

Abstract

Heritable disorders of connective tissue often predispose patients to aortic pathology and in particular aortic dissection. The Marfan syndrome, vascular Ehlers-Danlos syndrome, familial forms of thoracic aortic aneurysms or aortic dissection, and bicuspid aortic valve are all examples of heritable disorders that have associated defects affecting the integrity of the aortic wall, posing a risk of both aneurysmal dilation and dissection. The purpose of this review was to outline the phenotypes of the heritable syndromes that predispose to aortic dissection, present a guideline to their management and surveillance, and to offer insight into some of the surgical pitfalls that occur when repairing ascending aortic dissections in these types of patients.