Chen Yu, Ma Wei-Guo, Li Jian-Rong, Zheng Jun, Liu Yong-Min, Zhu Jun-Ming, Sun Li-Zhong
Department of Cardiovascular Surgery, Beijing Anzhen Hospital, Capital Medical University, and Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China.
Fu Wai Hospital and Cardiovascular Institute, Chinese Academy of Medical Sciences, Beijing 100037, China.
Ann Cardiothorac Surg. 2020 May;9(3):197-208. doi: 10.21037/acs.2020.03.10.
Chronic type A aortic dissection (cTAAD) in Marfan syndrome (MFS) is rare. Surgical experience is limited and the role of frozen elephant trunk (FET) technique remains undefined. We seek to evaluate the safety and efficacy of the total arch replacement (TAR) and FET technique for cTAAD in MFS.
The clinical data of sixty-eight patients with MFS undergoing FET and TAR for cTAAD were analyzed.
Mean age was 35.8±9.7 years and thirty-nine were male (57.4%). Operative mortality was 10.3% (7/68). Stroke occurred in one (1.5%), re-exploration for bleeding in five (7.3%), low cardiac output in four (5.9%), and acute renal failure in two (2.9%). Follow-up was complete in 100% (61/61) at mean 7.3±4.0 years. The false lumen was obliterated in 73.5% across FET and 50.0% in unstented descending aorta (DAo). Distal dilation occurred in twenty patients, six of whom underwent thoracoabdominal aortic replacement, one abdominal aortic replacement and one thoracic endovascular aortic repair (TEVAR). Late death occurred in five. At ten years, 59.8% were free from distal aortic dilation, and the incidences were 23.2% for death, 14.4% for distal reoperation, and 62.4% for reoperation-free survival. Predictors for operative mortality were extra-anatomic bypass [odds ratio (OR), 229.592; P=0.036], preoperative maximal size (DMax) of aortic sinuses (mm) (OR, 1.134; P=0.032) and cardiopulmonary bypass (CPB) time (minute) (OR, 1.061; P=0.041). Risk factors for aortic dilatation included patent false lumen at diaphragmatic hiatus [hazard ratio (HR), 5.374; P=0.008], preoperative DMax (mm) of proximal DAo (HR, 1.068; P=0.001) and renal arteries (HR, 1.102; P=0.005) which also predicted distal reoperation (HR, 1.149; P=0.001). The time from onset to operation (day) (HR, 1.002; P=0.004) and CPB time (minute) (HR, 1.032; P=0.036) predicted late death.
This study shows that the TAR and FET technique is a safe and durable approach to cTAAD in patients with MFS. The operation should be performed as early as possible to optimize clinical outcomes.
马方综合征（MFS）合并慢性A型主动脉夹层（cTAAD）较为罕见。手术经验有限，且冷冻象鼻（FET）技术的作用尚不明确。我们旨在评估全弓置换（TAR）联合FET技术治疗MFS合并cTAAD的安全性和有效性。
分析68例接受FET联合TAR治疗cTAAD的MFS患者的临床资料。
平均年龄为35.8±9.7岁，男性39例（57.4%）。手术死亡率为10.3%（7/68）。1例（1.5%）发生卒中，5例（7.3%）因出血再次手术，4例（5.9%）出现低心排，2例（2.9%）发生急性肾衰竭。100%（61/61）患者获得完整随访，平均随访时间为7.3±4.0年。FET段假腔闭合率为73.5%，未置入支架的降主动脉（DAo）假腔闭合率为50.0%。20例患者出现远端扩张，其中6例行胸腹主动脉置换，1例行腹主动脉置换，1例行胸段血管腔内修复术（TEVAR）。5例患者发生晚期死亡。10年时，59.8%患者无远端主动脉扩张，死亡率为23.2%，远端再次手术率为14.4%，无再次手术生存率为62.4%。手术死亡的预测因素为解剖外旁路[比值比（OR），229.592；P=0.036]、术前主动脉窦最大直径（DMax）（mm）（OR，1.134；P=0.032）和体外循环（CPB）时间（分钟）（OR，1.061；P=0.041）。主动脉扩张的危险因素包括膈肌裂孔处假腔通畅[风险比（HR），5.374；P=0.008]、术前近端DAo的DMax（mm）（HR，1.068；P=0.001）和肾动脉（HR，1.102；P=0.005），这些因素也可预测远端再次手术（HR，1.149；P=0.001）。发病至手术时间（天）（HR，1.002；P=0.004）和CPB时间（分钟）（HR，1.032；P=0.036）可预测晚期死亡。
本研究表明，TAR联合FET技术是治疗MFS合并cTAAD安全、持久的方法。应尽早手术以优化临床结局。
