Liu Hao, Chen Suwei, Luo Congcong, Zhong Yongliang, Qiao Zhiyu, Sun Lizhong, Zhu Junming
Department of Cardiovascular Surgery, Beijing Aortic Disease Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
Department of Thoracic Surgery, Shanghai Ninth People's Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.
Front Cardiovasc Med. 2023 Jun 28;10:1186181. doi: 10.3389/fcvm.2023.1186181. eCollection 2023.
The aortic root is the most frequent segment involved in Marfan syndrome. However, Marfan syndrome is a systemic hereditary connective tissue disorder, and knowledge regarding the outcomes of the native distal aorta after prophylactic aortic root surgery is limited.
From April 2010 to December 2020, 226 patients with Marfan syndrome and 1,200 patients without Marfan syndrome who underwent Bentall procedures were included in this study. By propensity score matching, 134 patients were assigned to each group. Clinical manifestations and follow-up data were acquired from hospital records and telephone contact. The cumulative incidence of aortic events was estimated in Marfan and non-Marfan patients with death as a competing risk.
Patients with and without Marfan syndrome had similar baseline characteristics after propensity score matching. Differences in the aortic root (62.25 ± 11.96 vs. 54.03 ± 13.76, < .001) and ascending aorta (37.71 ± 9.86 vs. 48.16 ± 16.01, < .001) remained after matching. No difference was observed in the frequency of aortic adverse events between the two groups (10.5% vs. 4.6%, = 0.106). The cumulative incidence of aortic events was not different between Marfan and non-Marfan patients (15.03% ± 4.72% vs. 4.18% ± 2.06%, = 0.147). Multivariate Cox regression indicated no significant impact of Marfan syndrome on distal aortic events (HR: 1.172, 95% CI: 0.263-5.230, = 0.835). Descending and abdominal aortic diameter above normal at the initial procedure were associated with the risk of distal aortic events (HR: 20.735, = .003, HR: 22.981, = .002, respectively).
New-onset events of the residual aorta in patients undergoing Bentall procedures between the Marfan and non-Marfan groups were not significantly different. Distal aortic diameter above normal at initial surgery was associated with a higher risk of adverse aortic events.
主动脉根部是马凡综合征最常累及的节段。然而，马凡综合征是一种全身性遗传性结缔组织疾病，关于预防性主动脉根部手术后主动脉远端天然节段的转归的知识有限。
2010年4月至2020年12月，本研究纳入了226例接受Bentall手术的马凡综合征患者和1200例非马凡综合征患者。通过倾向评分匹配，每组分配134例患者。从医院记录和电话联系中获取临床表现和随访数据。以死亡作为竞争风险，估计马凡综合征患者和非马凡综合征患者主动脉事件的累积发生率。
倾向评分匹配后，有和没有马凡综合征的患者具有相似的基线特征。匹配后，主动脉根部（62.25±11.96 vs. 54.03±13.76，<0.001）和升主动脉（37.71±9.86 vs. 48.16±16.01，<0.001）仍存在差异。两组间主动脉不良事件的发生率无差异（10.5% vs. 4.6%，P = 0.106）。马凡综合征患者和非马凡综合征患者主动脉事件的累积发生率无差异（15.03%±4.72% vs. 4.18%±2.06%，P = 0.147）。多因素Cox回归表明马凡综合征对主动脉远端事件无显著影响（HR：1.172，95%CI：0.263 - 5.230，P = 0.835）。初次手术时降主动脉和腹主动脉直径高于正常与主动脉远端事件风险相关（HR分别为：20.735，P = 0.003；22.981，P = 0.002）。
马凡综合征组和非马凡综合征组接受Bentall手术患者残余主动脉的新发事件无显著差异。初次手术时主动脉远端直径高于正常与主动脉不良事件风险较高相关。
