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

BACKGROUND: A 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. METHODS: Data 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. RESULTS: The 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. CONCLUSIONS: For 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.