Early and long-term results of a valve-sparing operation for Marfan syndrome

Background-We have previously described the experience, rationale, and deve lopment of a valve preserving technique, but its role in patients with Marf an syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of thi s operation in patients with Marfan syndrome. Methods and Results-Since 1979, 82 patients (73.2% of all patients with Mar fan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33. 9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no earl y deaths in 67 patients operated on electively. Actuarial survival for pati ents operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63.6%, and 63 .6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probab ility of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 yea rs. There were no instances of infective endocarditis or thromboembolic com plications except in 2 patients operated on early in the series who had cus p extension. At the end of the follow-up, trivial or no aortic regurgitatio n was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe i n 0. Conclusions-Valve-sparing operations are feasible in most patients with Mar fan syndrome; they are applicable to patients with both dissection and chro nic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warrante d in some cases.