[Sudden death and cardiovascular complications in Marfan syndrome: impact of surgical intervention].
Marfan syndrome is an autosomal dominant disorder of connective tissue characterized by abnormalities involving the skeletal, ocular, and cardiovascular systems. The cardiovascular complications of the syndrome lead to a reduced life expectancy for affected individuals if left untreated. Major cause of death include acute aortic dissection, aortic rupture, and sudden death, which resulted from congenital vascular fragility. Such life-threatening complications in Marfan syndrome can be managed effectively, by routine aortic imaging, beta-adrenergic blockade, and prophylactic replacement of the aortic root before the diameter exceeds 5.0 to 5.5 mm. Valve preserving aortic root reconstruction yielded improved postoperative quality of life compared with Bentall operation, by reducing late complications related to anticoagulants. It should be carried out before onset of aortic regurgitation for long-term native valve durability.
Department of Thoracic and Cardiovascular Surgery, Saga Meidical School.
Satoshi
S
Ohtsubo
Tsuyoshi
T
Itoh
D002318:Cardiovascular Diseases / Q000209:etiology* / Q000601:surgery*; D016757:Death, Sudden, Cardiac; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications*