Marfan and other connective tissue disorders: conservative and surgical considerations

Several inheritable disorders of connective tissue have clinical manifestations in the cardiovascular system. Of these, the most significant disorders are Marfan syndrome and Ehlers-Danlos syndrome type IV. The cardiovascular complication typically seen in Marfan syndrome is aneurysmal dilatation of the aortic root, which begins in the first or second decade of life and leads to premature death caused by aortic rupture, dissection, or cardiac complications of aortic regurgitation. However, the remaining aorta and its branches are vulnerable to subsequent aneurysmal degeneration, especially when involved with chronic dissection. Ehlers-Danlos syndrome type IV is characterized by extremely friable arteries that are prone to aneurysm and rupture and are uncommonly difficult to repair. The authors review the literature on these disorders and document their personal experience with their vascular complications, with emphasis on their experience with the aortic tailoring procedure in dissection of the descending thoracic aorta.