Outcomes of endovascular therapy for Stanford type B aortic dissection in patients with Marfan syndrome
Outcomes of endovascular therapy for Stanford type B aortic dissection in patients with Marfan syndrome
复制标题
马凡综合征患者斯坦福 B 型主动脉夹层血管内治疗的结果
DOI：
10.1016/j.jtcvs.2021.05.045
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发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Dong Z
中科院分区：
文献类型：
--
作者：
Jiang X;Chen B;Jiang J;Shi Y;Ma T;Fu W;Dong Z
目的评价马凡综合征（MFS） Stanford B型主动脉夹层（TBAD）患者行胸血管内主动脉修复术（TEVAR）的中期疗效。方法在2009年1月至2019年12月期间，纳入了因TBAD而接受TEVAR治疗的MFS患者。收集和分析人口统计资料、术前和围手术期临床资料及随访资料。用Kaplan-Meier分析计算累计生存率和无再干预率。结果共纳入26例患者。平均年龄38.5±10.7岁（24 ~ 64岁）。住院死亡率为0。5年的累积生存率为88.1%(95%可信区间[CI]， 67.5%-98.5%)， 10年的累积生存率为82.9% （95% CI, 60.2%-93.3%）。假腔沿支架长度形成的患者的累积生存率（P< 0.05）和再干预自由度（P= 0.01）明显高于未通畅假腔患者。5年再干预自由度为83.4% (95% CI, 61.4% ~ 93.4%)， 10年再干预自由度为50.3% （95% CI, 21.4% ~ 97.3%）。有和未做过主动脉根部手术的患者的再干预自由度无显著差异（P= 0.46）。结论stevar治疗MFS患者TBAD是安全有效的。围手术期死亡率和发病率低；然而，终身密切随访的临床和影像学是强制性的，以防止主动脉破裂。未闭FL患者晚期死亡风险高。
ObjectiveTo evaluate the mid-term outcomes of thoracic endovascular aorta repair (TEVAR) for Stanford type B aortic dissection (TBAD) in patients with Marfan syndrome (MFS).MethodsBetween January 2009 and December 2019, patients with MFS who underwent TEVAR for TBAD were enrolled. Demographic data, preoperative and perioperative clinical profiles, and follow-up data were collected and analyzed. The cumulative survival and freedom from reintervention rates were calculated with Kaplan-Meier analysis.ResultsA total of 26 patients were enrolled. The mean age was 38.5 ± 10.7 (range, 24-64 years). The in-hospital mortality was 0. The cumulative survival rate was 88.1% (95% confidence interval [CI], 67.5%-98.5%) at 5 years and 82.9% (95% CI, 60.2%-93.3%) at 10 years. Patients with a thrombosed false lumen (FL) along the length of the stent had a significantly higher cumulative survival rate (P< .05) and freedom from reintervention (P= .01) than patients with patent FL. The freedom from reintervention was 83.4% (95% CI, 61.4% to 93.4%) at 5 years and 50.3% (95% CI, 21.4%-97.3%) at 10 years. There was no significant difference freedom from reintervention in freedom from reintervention between patients with and those without a previous aortic root procedure (P= .46).ConclusionsTEVAR can be performed safely and effectively for TBAD in patients with MFS. Perioperative mortality and morbidity were low; however, lifelong close follow-up in the clinic and imaging are mandatory to prevent aortic rupture. Patients with a patent FL were at high risk of late death.