• C Detter, H Mair, H G Klein, C Georgescu, A Welz, and B Reichart.
• Department of Cardiac Surgery, University Hospital Grosshadern, Ludwig-Maximilian-University, Munich, Germany.
• Eur J Cardiothorac Surg. 1998 Apr 1;13(4):416-23.

ObjectiveAortic aneurysms and dissections are the leading causes of premature death in Marfan syndrome (MfS). This study aims to compare long-term results of surgically treated aortic aneurysms and dissections in patients with and without MfS in respect to early and late prognosis.MethodsFrom March 1975 to August 1994, 33 patients with classic MfS (group A, age 34.2 +/- 9 years) and 298 patients with non-fibrillinopathic aortic disease (group B, age 54 +/- 13 years) underwent aortic surgery. Acute dissections occurred in 57.6 (A) versus 37.9% (B). A total of 54.6% of patients in group A were treated with a composite graft versus 16.4% in B. The aortic arch and the descending aorta was replaced in 30.4% of MfS patients and 24.9% of patients without MfS.ResultsWe observed 7 (25.0%, A) versus 35 (14.2%, B) late deaths among the 28 (A) versus 247 (B) early survivors. In 5 patients (17.9%) of A and 8 patients (3.2%) of B, late death was caused by redissection or recurrent aneurysm (P < 0.001). Long-term survival after 5, 10 and 15 years in group A was 82 +/- 7, 60 +/- 11 and 30 +/- 22%, and 75 +/- 3, 69 +/- 3 and 64 +/- 4% in group B. A total of 22 reoperations were performed in 11 MfS patients, 17 reoperations were due to recurrent aortic diseases. Three of the 8 patients underwent reoperation after Wheat procedure because of sinus valsalva aneurysm. None of the patients with composite graft replacement needed reoperation in this segment, but 3 patients suffered from redissection at the proximal aortic arch. In group B, reoperations were significantly less frequent (10.7%) compared to MfS patients (66.7%; P < 0.001).ConclusionsSurgical treatment of aortic disease in MfS patients is associated with a high risk of redissection and recurrent aneurysm. If the ascending aorta needs to be replaced, we recommend the composite graft technique and a more aggressive approach to reduce the frequency of distal reoperations. In order to reduce the high reoperation rate in MfS patients, frequent clinical follow-up may contribute to improve life expectancy in MfS patients.

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