Zhang Boyao, Xue Qing, Tang Yangfeng, Yu Shangyi, Fan Xingli, Xu Zhiyun, Han Lin
Department of Cardiovascular Surgery, Changhai Hospital, Naval Medical University (Second Military Medical University), Shanghai, China.
J Thorac Dis. 2020 Dec;12(12):7106-7116. doi: 10.21037/jtd-20-2109.
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.
We retrospectively analyzed the results of hospitalization and long-term follow-up of MFS patients who underwent surgical intervention in our center.
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).
The David procedure was the most beneficial and had the highest long-term patient survival rates.
大多数马凡综合征（MFS）患者患有胸主动脉疾病，这是主要的死亡原因。本研究的目的是分析不同手术方式对MFS患者预后的影响。
我们回顾性分析了在本中心接受手术干预的MFS患者的住院及长期随访结果。
135例MFS患者中，11例在住院期间死亡（8.1%）。近端手术组和远端手术组的院内死亡率无统计学差异（P = 0.11）。与接受近端主动脉手术的患者相比，接受主动脉弓和远端手术的患者术后更易出现呼吸功能障碍（P = 0.008）。手术方式与住院期间并发症的发生率无关。术前纽约心脏协会（NYHA）心功能分级IV级（P = 0.047）、射血分数（EF）<50%（P = 0.047）、术前心房颤动（P = 0.042）以及夹层累及冠状动脉（P = 0.02）是术后死亡的独立危险因素。随访15年后，David组无死亡病例，而Bentall组患者的15年生存率为73%±13.5%，主动脉弓手术组患者为71%±13.9%（P = 0.42）。David组患者15年后无需再次手术的概率为58.9%±20%，Bentall组患者为58.7%±12.1%，Bentall + 主动脉弓组患者为71.5%±10.5%，主动脉弓 + 支架组患者为12.5%±11.7%（P = 0.007）。
David手术最为有益，患者长期生存率最高。
