Reoperation in Cardiovascular Surgery for Marfan Syndrome

Abstract

Without surgical treatment, the life expectancy of patients with Marfan’s syndrome is reduced by the associated cardiovascular lesions. In this study, we reviewed our experiences of the patients with Marfan’s syndrome who required multiple surgical procedures to identify the optimal treatment for these patients. Twenty-two patients with Marfan’s syndrome were operated on at our division of cardiovascular surgery. Among them, 9 patients (41.0%) underwent multiple surgical procedures. There was a mean age of 45.4±12.3 years at initial surgery. The causes of additional procedures were new dissection in 7, progression of aortic valve regurgitation (AR) in 2, coronary false aneurysm in 1, progression of annulo aortic ectasia + AR + mitral valve regurgitation + new dissection in 1, enlargement of residual thoracoabdominal aortic aneurysm in 1. Among them, 2 patients were operated on multiple additional procedures (three to four times), and were finally total aortic replacements. Additional procedures of the remaining 7 patients were total arch or hemiarch replacement + Bentall’s procedures in 2, Bentall’s procedures in 2, aortic valve replacement in 1, descending aortic replacement in 1, total arch replacement in 1. There was one early death (11.1%) due to graft infection. In 9 patients, both ascending and descending aorta were replacement. Among them, 5 patients ultimately underwent total arch replacement. The observed 10- year survival rate was 64.3% and freedom from reoperation was 37.5%. In conclusion, total arch replacement with an elephant trunk at the initial operation should be performed in order to minimize the future risk of vascular events and to eliminate the need for extensive replacement in a reoperation. Although a strategy of this procedure which is associated with a high level of risk, we believe that these extensive procedures will be decreased in a reoperation. It is anticipated that further improvement in freedom from reoperation of patients with Marfan’s syndrome will be observed in late results.