455 Cardiovascular Reoperations in Marfan Syndrome Vedat Erentug, M.D., ∗ Adil Polat, M.D., ∗ Nilgun Ulusoy Bozbuga, M.D.,† Ebru Polat, M.D., ∗ Hasan Basri Erdogan, M.D., ∗ Kaan Kirali, M.D.,† Mustafa Guler, M.D.,† Esat Akinci, M.D.,‡ and Cevat Yakut, M.D.‡ ∗ Cardiovascular Surgeon; †Associate Professor in Cardiovascular Surgery; ‡Surgeon-in-Chief, Associate Professor in Cardiovascular Surgery, Ko¸ suyolu Heart and Research Hospital, ˙ Istanbul, Turkey ABSTRACT Background and aim of the study: The purpose of this study is to analyze the outcome results of reoperations in Marfan syndrome patients. Methods: Between 1985 and December 2004, 49 patients with Marfan syndrome were operated for aortic aneurysms. Of these 49 patients, 9 (18,4%) required ≥1 reopera- tions after a mean duration of 32.2 ± 26.6 months. The mean duration of follow-up was 52.0 ± 46.8 months, a total of 39 patient/years. Survival free of reoperation was calculated by Cox regression analysis. Results: Surgical indication for operation was a chronic aneurysmal dilatation of the ascending or abdominal aorta in seven patients (77.8%) and aortic dissection in two (22.2%) at the initial operation. In the reoperations, repair of thoracoabdominal aortic aneurysm with separated graft interposition in six patients (66.7%), re- placement of ascending aorta in one (11.1%), replacement of ascending and hemiarchus aorta in one (11.1%), and mitral valve replacement in three patients (33.3%) were performed. The hospital mortality was 11.1% with one patient. Among the survivors, one expired in the follow-up five months after the second opera- tion (12.5%). With the Cox regression analysis, survival without reoperation for 13, 24, and 123 months are 95.56 ± 3.04%, 90.66 ± 4.40%, and 60.32 ± 12.63%, respectively. Mean survival for reoperated patients is 99 ± 14 months (95% confidence interval 72–127 months). Conclusions: Reoperations can be done with low morbidity and mortality. Patients should be kept under close follow-up using imaging techniques infinitely. doi: 10.1111/j.1540-8191.2006.00276.x (J Card Surg 2006;21:455-457) Marfan syndrome is an autosomal dominant disor- der characterized by the deficiency of fibrillin-1 protein. Disease has a progressive nature with many different organ systems involvement. Cardiovascular complica- tions are of special importance as they constitute the primary cause of mortality. 1 Aortic involvement is fa- tal unless operated. Last decade showed us the mi- tral valve–related complications also should be of no- tice, especially for the pediatric group of patients. 2,3 Introduction of aortic root replacement with composite valve graft opened a new era for Marfan patients and caused a considerable increase in survival. We have been using a flanged modification of Bentall procedure since 1995. 4 The median survival for Marfan patients is estimated to be 61 years. 5 The increase in survival brings out new problems for Marfan patients. The progressive nature of the disease affects both survival and life quality of the patients. New onset dissections and aneurysms of the aorta proximal or distal to the previously implanted grafts, progression in mitral regurgitation in patients with prolapsed valves are seen in outpatient clinics and This paper was presented in The Asian Society for Cardiovascular Surgery (ASCVS) 12th Annual Meeting ˙ Istanbul, T ¨ urkiye, April 22, 2004. Address for correspondence: Vedat Erentug, M.D., Ulus Vadi Konutları , A1/9 84040 Ulus, Besı ktas, Istanbul, Turkey. Fax: +90 212 249 40 78; e-mail: drvedat2002@yahoo.com routine controls more often. We should consider, there- fore, the necessity of reoperative procedures from the time of initial diagnosis in clinical decision-making in Marfan patients. In search for a more detailed data for these Marfan patients, we evaluated the reoperative procedures and the characteristics of these patients. MATERIALS AND METHODS Between 1985 and June 2004, 49 patients with Mar- fan syndrome were operated on for aortic aneurysms with or without dissection. Diagnosis of Marfan syn- drome was made according to the Ghent nosology. 6 Of these 49 patients, nine (18.4%) required one or more reoperations after a mean duration of 32.2 ± 26.6 months between the operations. In the group of reoperated patients, six were male and three were female (male to female ratio 2:1). Nine patients had 22 operations with a mean inter- val of 32.3 ± 26.6 months (range 10–88 months) be- tween operations. Of these 22 procedures, only one was on an emergent basis. At the reoperations, the most common indication was the presence of an aor- tic aneurysm, which had occurred in seven of these nine patients (77.7%). Indications are aortic aneurysm in nine operations (40.9%), acute aortic dissection in four (18.2%), chronic aortic dissection in four (18.2%), chronic dissection and aneurysm of aorta in two (9.1%)