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Eur J Cardiothorac Surg 1998;13:416-423
© 1998 Elsevier Science NL

Long-term prognosis of surgically-treated aortic aneurysms and dissections in patients with and without Marfan syndrome

^a Department of Cardiac Surgery, University Hospital Großhadern, Ludwig-Maximilian-University, Marchioninistraße 15, 81366 Munich, Germany
^b Department of Clinical Chemistry, University Hospital Großhadern, Ludwig-Maximilian-University, Marchioninistraße 15, 81366 Munich, Germany

Received 25 August 1997; received in revised form 2 February 1998; accepted 10 February 1998.

Corresponding author. Present address: Department of Thoracic and Cardiovascular Surgery, Johann Wolfgang Goethe University, Theodor Stern-Kai 7, 60590 Frankfurt, Germany. Tel.: +49 69 63015850; fax.+49 69 63015849.

Objective: Aortic 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. Methods: From 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. Results: We 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). Conclusions: Surgical 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.

Key Words: Marfan syndrome • Aortic aneurysm • Redissection • Fibrillin • Connective tissue

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