Aortic dissection in Marfan's syndrome

Background. Aortic dissection is the most frequent cause of premature death in Marfan's syndrome. Low-risk elective surgery of the abnormal aortic roo t has the potential to prevent this complication. Methods. We examine genetic, structural, and pathophysiological mechanisms of aortic dissection and discuss the surgical methods used when dissection occurs. Results. Abnormal fibrillin disturbs the functional relationship between bl ood flow and vascular endothelial cell response (mechanotransduction). Decr eased arterial distensibility also decreases aortic wall stress, thereby pr edisposing to dissection in the weakened arterial wall. Radical root and wa ll surgery and lifelong beta-blockade are required after aortic dissection. Conclusions. Detailed lifelong medical and surgical treatment can greatly p rolong life in Marfan's syndrome. Elective aortic root replacement is param ount in preventing aortic dissection and avoiding subsequent problems in th e distal aorta. (C) 1999 by The Society of Thoracic Surgeons.