S Westaby1. 1. The Oxford Heart Centre, John Radcliffe Hospital, England.
Abstract
BACKGROUND: Aortic dissection is the most frequent cause of premature death in Marfan's syndrome. Low-risk elective surgery of the abnormal aortic root 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 blood flow and vascular endothelial cell response (mechanotransduction). Decreased arterial distensibility also decreases aortic wall stress, thereby predisposing to dissection in the weakened arterial wall. Radical root and wall surgery and lifelong beta-blockade are required after aortic dissection. CONCLUSIONS: Detailed lifelong medical and surgical treatment can greatly prolong life in Marfan's syndrome. Elective aortic root replacement is paramount in preventing aortic dissection and avoiding subsequent problems in the distal aorta.
BACKGROUND: Aortic dissection is the most frequent cause of premature death in Marfan's syndrome. Low-risk elective surgery of the abnormal aortic root 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 blood flow and vascular endothelial cell response (mechanotransduction). Decreased arterial distensibility also decreases aortic wall stress, thereby predisposing to dissection in the weakened arterial wall. Radical root and wall surgery and lifelong beta-blockade are required after aortic dissection. CONCLUSIONS: Detailed lifelong medical and surgical treatment can greatly prolong life in Marfan's syndrome. Elective aortic root replacement is paramount in preventing aortic dissection and avoiding subsequent problems in the distal aorta.