LONG-TERM PROGNOSIS OF SURGICALLY-TREATED AORTIC-ANEURYSMS AND DISSECTIONS IN PATIENTS WITH AND WITHOUT MARFAN-SYNDROME

Objective: Aortic aneurysms and dissections are the leading causes of premature death in Marfan syndrome (MfS), This study aims re, compare long-term results of surgically treated aortic aneurysms and dissectio ns in patients with and without MIS in respect to early and late progn osis. Methods: From March 1975 to August 1994, 33 patients with classi c MfS (group At age 34.2 +/- 9 years) and 298 patients with non-fibril linopathic aortic disease (group B, age 54 +/- 13 years) underwent aor tic 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. R esults: We observed 7 (25.0%, A) versus 35 (14.2%, B) late deaths amon g 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 p erformed in 11 MfS patients, 17 reoperations were due to recurrent aor tic diseases. Three of the 8 patients underwent reoperation after Whea t procedure because of sinus valsalva aneurysm. None of the patients w ith composite graft replacement needed reoperation in this segment: bu t 3 patients suffered from redissection at the proximal aortic arch. I n group B, reoperations were significantly less frequent (10.7%) compa red to MfS patients (66.7%, P < 0.001), Conclusions: Surgical treatmen t of aortic disease in MfS patients is associated with a high risk of redissection and recurrent aneurysm. If the ascending aorta needs to b e replaced, we recommend the composite graft technique and a more aggr essive approach to reduce the frequency of distal reoperations. In ord er to reduce the high reoperation rate in MfS patients, frequent clini cal follow-up may contribute to improve life expectancy in MfS patient s. (C) 1998 Elsevier Science B.V. All rights reserved.