Efficacy of cardiovascular surgery for Marfan syndrome patients: a single-center 15-year follow-up study

J Thorac Dis. 2020 Dec;12(12):7106-7116. doi: 10.21037/jtd-20-2109.

Abstract

Background: Most Marfan syndrome (MFS) patients have thoracic aortic diseases which is the major cause of death. The aim of the study is to analyze the impact of different surgical procedures on prognosis of MFS patients.

Methods: We retrospectively analyzed the results of hospitalization and long-term follow-up of MFS patients who underwent surgical intervention in our center.

Results: Of the 135 MFS patients, 11 died during hospitalization (8.1%). There were no statistical differences in in-hospital mortality between the proximal surgery group and the distal surgery group (P=0.11). Compared to patients who underwent proximal aortic surgery, patients who underwent arch and distal surgery were more likely to have postoperative respiratory dysfunction (P=0.008). The type of surgical procedure was not associated with the incidence of complications during hospitalization. Pre-surgical New York Heart Association (NYHA) Functional Classification IV (P=0.047), EF <50% (P=0.047), pre-surgical atrial fibrillation (P=0.042), and the injury of dissection propagating onto coronary arteries (P=0.02) were independent risk factors for post-surgical mortality. After 15 years of follow-up, there were no deaths in the David group, while the 15-year survival rate for patients in the Bentall group was 73%±13.5%, and 71%±13.9% for patients in the arch surgery group (P=0.42). The probability of patients in the David group not requiring re-surgery after 15 years was 58.9%±20%, while it was 58.7%±12.1% for patients in the Bentall group, 71.5%±10.5% for patients in the Bentall + Arch group, and 12.5%±11.7% for patients in the Arch + Stent group (P=0.007).

Conclusions: The David procedure was the most beneficial and had the highest long-term patient survival rates.

Keywords: Marfan syndrome (MFS); aortic dissection; cardiovascular surgery.