ABSTRACT

The natural of aneurysms involving the ascending aorta is to progressively enlarge over time, leading to an ascending aortic dissection in the absence of prophylactic repair of the diseased segment of the aorta. Therefore, thoracic aortic aneurysms and ascending aortic dissections are related conditions, termed thoracic aortic aneurysms and dissections (TAAD) for this chapter. It is well established that known genetic syndromes predispose to TAAD, such as Marfan syndrome (MFS). This chapter will review genetic syndromes that are associated with TAAD, including MFS and Loeys-Dietz aortic syndrome (LDAS).