Long-term outcomes after aortic root replacement for patients with Marfan syndrome.

Abstract

BackgroundA diversity of surgical strategies are used to treat Marfan syndrome patients with aortic disease. We sought to evaluate the long-term efficiency of aortic root replacement (ARR) for patients with Marfan syndrome.MethodsData were collected from 131 patients with Marfan syndrome and aortic disease who underwent ARR in our center. We retrospectively analyzed the long-term outcomes of these patients, among whom 68 had been diagnosed with aortic aneurysm (AA) and had undergone ARR. The remaining 63 patients had aortic dissection (AD); of these, 35 underwent ARR for limited ascending AD, while the others underwent ARR and total arch replacement combined with frozen elephant trunk (FET). Risk factors for survival and reoperation were identified.ResultsThe operative mortality rate was 4.58%. Age >40 years was the sole risk factor for operative mortality. During follow-up, 12 deaths occurred. Patients aged <25 years and female patients were more prone to late death than were other patients. Indications for reoperation were noted in 22 patients, and the risk factors were age <30 years and female sex. At 5 years, the survival rate was 92.96%, and the freedom from reoperation rate was 88.36%. At 10 years, the survival rate was 85.25%, and the freedom from reoperation rate was 71.75%. The survival and freedom from reoperation rates were significantly higher in patients with AD than in those with AA. Specifically, chronic AD was a greater risk factor for late survival than was acute AD in patients with Marfan syndrome.ConclusionsFor patients with Marfan syndrome and aortic disease, ARR can be safely performed and results in low operative mortality and favorable long-term survival. Young and female patients have a higher risk for late death and reoperation. To prevent AD, surgical intervention should be promptly implemented following the diagnosis of aortic sinus dilation.