马凡综合征患者行 Frozen Elephant Trunk 手术治疗 Stanford A 型主动脉夹层的长期转归。
Long-term outcomes of frozen elephant trunk for type A aortic dissection in patients with Marfan syndrome.
机构信息
Department of Cardiovascular Surgery, Beijing Aortic Disease Center, Beijing Anzhen Hospital of Capital Medical University, Beijing Institute of Heart, Lung and Blood Vessel Diseases, and Beijing Engineering Research Center of Vascular Prostheses, Beijing, China; Fu Wai Hospital and Cardiovascular Institute, Chinese Academy of Medical Sciences, Beijing, China; Aortic Institute at Yale-New Haven, Yale University School of Medicine, New Haven, Conn.
Department of Cardiovascular Surgery, Beijing Aortic Disease Center, Beijing Anzhen Hospital of Capital Medical University, Beijing Institute of Heart, Lung and Blood Vessel Diseases, and Beijing Engineering Research Center of Vascular Prostheses, Beijing, China.
出版信息
J Thorac Cardiovasc Surg. 2017 Oct;154(4):1175-1189.e2. doi: 10.1016/j.jtcvs.2017.04.088. Epub 2017 Jun 16.
OBJECTIVE
The use of the frozen elephant trunk (FET) technique for repair of type A aortic dissection (TAAD) in Marfan syndrome (MFS) is controversial. We seek to evaluate the efficacy of FET and total arch replacement (TAR) for TAAD in patients with MFS.
METHODS
The early and long-term outcomes were analyzed for 106 patients with MFS (mean age, 34.5 ± 9.7 years) undergoing FET + TAR for TAAD.
RESULTS
Operative mortality was 6.6% (7 of 106). Spinal cord injury and stroke occurred in 1 patient each (0.9%), and reexploration for bleeding occurred in 6 patients (5.7%). Extra-anatomic bypass was the sole risk factor for operative mortality and morbidity (odds ratio [OR], 7.120; 95% confidence interval [CI], 1.018-49.790; P = .048). Follow-up was complete in 97.0% (96 of 99), averaging 6.3 ± 2.8 years. Late death occurred in 17 patients. Patients with acute TAAD were less prone to late death than those with chronic TAAD (OR, 0.112; 95% CI, 0.021-0.587; P = .048). Twelve patients required late reoperation, including thoracoabdominal aortic repair in 8, thoracic endovascular aortic repair for distal new entry in 3, and coronary anastomotic repair in 1. At 5 years, survival was 86.6% (95% CI, 77.9%-92.0%) and freedom from reoperation was 88.8% (95% CI, 80.1%-93.4%), and at 8 years, survival was 74.1% (95% CI, 61.9%-83.0%) and freedom from reoperation was 84.2% (95% CI, 72.4%-91.2%). In competing risks analysis, mortality was 4% at 5 years, 18% at 8 years, and 25% at 10 years; the respective rates of reoperation were 10%, 15%, and 15%; and the respective rates of survival without reoperation were 86%, 67%, and 60%. Survival was significantly higher in patients who underwent root procedures during FET + TAR (P = .047). Risk factors for reoperation were days from diagnosis to surgery (OR, 1.160; 95% CI, 1.043-1.289; P = .006) and Bentall procedure (OR, 12.012; 95% CI, 1.041-138.606; P = .046).
CONCLUSIONS
The frozen elephant trunk and total arch replacement procedure can be safely performed for TAAD in MFS with low operative mortality, favorable long-term survival and freedom from reoperation. A concomitant Bentall procedure was predictive of better long-term survival and increased risk for late reoperation. These results argue favorably for the use of the FET + TAR technique in the management of TAAD in patients with MFS.
目的
对于马凡综合征（MFS）患者的A型主动脉夹层（TAAD），使用冷冻象鼻技术（FET）修复存在争议。我们旨在评估 FET 和全主动脉弓置换术（TAR）治疗 MFS 患者 TAAD 的疗效。
方法
分析了 106 例 MFS 患者（平均年龄 34.5±9.7 岁）接受 FET+TAR 治疗 TAAD 的早期和长期结果。
结果
手术死亡率为 6.6%（106 例中有 7 例）。1 例发生脊髓损伤，1 例发生卒中，6 例（5.7%）因出血再次探查。体外旁路是手术死亡率和发病率的唯一危险因素（比值比[OR]，7.120；95%置信区间[CI]，1.018-49.790；P=0.048）。97.0%（99 例中的 96 例）获得了完整的随访，平均随访时间为 6.3±2.8 年。17 例患者死亡。急性 TAAD 患者的晚期死亡率低于慢性 TAAD 患者（OR，0.112；95%CI，0.021-0.587；P=0.048）。12 例患者需要晚期再次手术，包括 8 例胸腹主动脉修复、3 例胸主动脉腔内修复术治疗远端新入口、1 例冠状动脉吻合口修复术。5 年时，生存率为 86.6%（95%CI，77.9%-92.0%），无再次手术率为 88.8%（95%CI，80.1%-93.4%），8 年时，生存率为 74.1%（95%CI，61.9%-83.0%），无再次手术率为 84.2%（95%CI，72.4%-91.2%）。在竞争风险分析中，5 年时死亡率为 4%，8 年时为 18%，10 年时为 25%；相应的再手术率为 10%、15%和 15%；无再手术生存率分别为 86%、67%和 60%。在接受 FET+TAR 期间进行根部手术的患者生存率显著更高（P=0.047）。再手术的危险因素包括从诊断到手术的天数（OR，1.160；95%CI，1.043-1.289；P=0.006）和 Bentall 手术（OR，12.012；95%CI，1.041-138.606；P=0.046）。
结论
对于 MFS 患者的 TAAD，冷冻象鼻和全主动脉弓置换术可安全实施，手术死亡率低，长期生存率和无再次手术率良好。同期 Bentall 手术预测长期生存率更高，晚期再手术风险增加。这些结果支持 FET+TAR 技术在 MFS 患者 TAAD 治疗中的应用。
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