Canadian journal of anaesthesia = Journal canadien d'anesthésie
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An 18-year-old male with Marfan's syndrome underwent surgery for repair of an ascending aortic dissection. The clinical features of this congenital syndrome and the acute management of its complications are described. As elective surgery is frequently required for ocular, musculo-skeletal or cardiovascular problems in people with Marfan's syndrome, patients must be carefully assessed preoperatively for specific complications. ⋯ The commonest causes of sudden death are cardiovascular complications, in particular rupture and dissection of the ascending aorta. Preoperative assessment should include echocardiography to determine the size of the aortic root. The anaesthetic technique chosen should both decrease myocardial contractility and avoid sudden increases in contractility, in order to minimise the risk of aortic dissection or rupture.