Frequency of reoperations in patients with marfan syndrome

Background: We undertook a retrospective study of the pattern of reoperations in surgical patients with Marfan disease. Methods: Between 1985 and 2008, 83 Marfan patients (60 males, 23 females) underwent 155 aortic operations in our institution. Twenty-eight patients had acute dissection (22 type A, 6 type B), and two had aortic rupture. Mean age at initial operation was 32 ± 13 years. Operations included valve-sparing or Bentall aortic root repair, and ascending aorta, arch, descending thoracic, thoracoabdominal aorta, and infrarenal aortic replacement. Sixty-one patients whose initial operation was elective (Group I) were compared with 22 patients with initial emergency surgery (Group II). Results: Overall, 81/83 patients ultimately underwent root/ascending repair: 64% initially and 36% at reoperation. Operative mortality in Group I was 1.6% for both initial operations and reoperations vs 9.0% and 0% in Group II. Significant differences between Group I and Group II patients included: total reoperations (1 vs 3, p = 0.05); arch operations (0 vs 1, p = 0.003); descending thoracic aortic operations (0 vs 0.5, p = 0.003); and total aortic segments replaced (1.6 ± 1.0 vs 2.4 ± 1.1, p = 0.001). Survival at 5 and 10 years did not differ between Group I and II patients (87% and 71% vs 82% and 56%, p = 0.19). Conclusions: Although reoperation occurs in about half of surgical Marfan patients, reoperative mortality is low. Patients with initial elective procedures fare better than those with initial emergency surgery: they have fewer subsequent operations, fewer aortic segments replaced, and trend toward improved survival. Elective root replacement should be seriously considered in any Marfan patient with significant root dilatation.