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 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.
Westaby
S
S
The Oxford Heart Centre, John Radcliffe Hospital, England.
D000784:Aortic Dissection / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D001011:Aorta / Q000473:pathology / Q000601:surgery*; D001014:Aortic Aneurysm / Q000209:etiology / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D004730:Endothelium, Vascular / Q000473:pathology; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome