Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.

Cardiovascular reoperations in Marfan syndrome.

Erentug V, Polat A, Bozbuga NU, Polat E, Erdogan HB, Kirali K, Guler M, Akinci E, Yakut C

Cardiovascular Surgeon, Koşuyolu Heart and Research Hospital, Istanbul, Turkey. drvedat2002@yahoo.com

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 > or =1 reoperations 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%), replacement 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 operation (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.

Published 4 September 2006 in J Card Surg, 21(5): 455-7.
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