• C Alexiou, S M Langley, P Charlesworth, M P Haw, S A Livesey, and J L Monro.
• Department of Cardiac Surgery, The General Hospital, Southampton, United Kingdom.
• Ann. Thorac. Surg. 2001 Nov 1; 72 (5): 1502-7; discussion 1508.

BackgroundThe purpose of this study was to evaluate the early and late clinical outcome after aortic root replacement (ARR) in patients with Marfan's syndrome.MethodsA total of 65 consecutive patients with Marfan's syndrome (mean age 41.7 +/- 10.7 years, range 15 to 76 years) undergoing ARR between 1972 and 1998 in Southampton were studied. Of the patients, 45 had a chronic aneurysm of the ascending aorta and 20 had a type A dissection (16 acute and 4 chronic). The operations were elective in 38 and nonelective in 27 cases (emergency in 22 and urgent in 5). Mean size of the ascending aorta was 6.3 +/- 1.4 cm (3.8 to 12 cm). A Bentall procedure was performed in 62 and a homograft root replacement in 3 patients. Mean follow-up was 8 +/- 4.1 years (0 to 22.9 years).ResultsOperative mortality was 6.1% (4 deaths) (for the elective vs nonelective procedures it was 2.6% vs 11%, p = 0.2). The 10-year freedom from thromboembolism, hemorrhage, and endocarditis was 88%, 89.8%, and 98.4% (0.9%, 0.9%, and 0.2% per patient-year) and from late aortic events it was 86.3% (1.3% per patient-year). Aortic root replacement for dissection was an independent predictor of occurrence of late aortic events (p = 0.01). Five patients had a reoperation with one early death. The 10-year freedom from reoperation was 89.2% (1.1% per patient year) (for elective and nonelective procedures, 90.8% vs 84.6%, p = 0.6). The 10-year survival, including operative mortality, was 72.7% (for elective and nonelective procedures, 78% vs 66.5%, p = 0.6). Late aortic events was an independent adverse predictor of survival (p = 0.02).ConclusionsIn patients with Marfan's syndrome, elective ARR, usually for chronic aneurysm, is associated with a low mortality, low rate of aortic complications, and good late survival. Nonelective ARR, mostly for dissection, has a greater operative risk and a significantly higher incidence of late catastrophic aortic events. Early prophylactic surgery in these patients is therefore recommended. Long-term clinical and radiologic follow-up to prevent or to treat late aortic events is highly desirable.

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