Pediatric Cardiac Surgery, New York Presbyterian/Columbia University Medical Center, Morgan Stanley Children's Hospital, New York, New York; Pediatric Cardiac Surgery, Cardiovascular Surgery, Heart Center Freiburg University, Freiburg, Germany; Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Pediatric Cardiac Surgery, Cardiovascular Surgery, Heart Center Freiburg University, Freiburg, Germany; Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Ann Thorac Surg. 2020 Feb;109(2):555-565. doi: 10.1016/j.athoracsur.2019.09.059. Epub 2019 Nov 9.
To characterize treatment of transposition of great arteries with ventricular septal defect and left ventricular outflow tract obstruction (LVOTO) in Germany and to analyze late outcomes.
German Registry for Congenital Heart Defects data were searched for transposition of great arteries with ventricular septal defect and LVOTO. One hundred thirty-nine patients were treated at 15 institutions between 1968 and 2016. Risk factor analyses were performed for the primary endpoints of death and reoperation/reintervention.
Follow-up was 88% complete, with a mean follow-up of 16 ± 7 years (median, 15 years [range, 1-48]) and a cumulative follow-up comprising 1739 patient-years. Atrial switches were performed in 15%, Rastelli procedure in 48%, Nikaidoh procedure in 9%, réparation a l'étage ventriculair (REV) in 8%, and arterial switch in 20%. Actuarial survival at 30 years was 86% (range, 83%-90%), with no difference between repairs. Freedom from primary reoperation was 17% (range, 12%-21%) at 30 years. Freedom from reoperation for LVOTO at 30 years was 74% (range, 70%-78%). Recurrent LVOTO was found in 9%, with risk factor diffuse subvalvular LVOTO (odds ratio, 9.8; P = .04). Late freedom from first reoperations other than right ventricle-to-pulmonary artery conduit was 60% (range, 59%-61%), with predictors multiple ventricular septal defect (hazard ratio, 6; P = .03) and Rastelli procedure (hazard ratio, 12; P = .03). Absolute reoperation rates were lowest for REV procedure (16% at a mean of 12 years).
Long-term survival is good without detectable differences between operations. Since the early 2000s increasing use of Nikaidoh and REV procedures was obvious, with REV performing particularly well. Surgery-specific and -unspecific reoperations are most common after the Rastelli technique.
描述德国治疗大动脉转位合并室间隔缺损和左心室流出道梗阻（LVOTO）的方法，并分析其远期结果。
在德国先天性心脏病注册研究中搜索大动脉转位合并室间隔缺损和 LVOTO 的患者数据。1968 年至 2016 年，15 家中心共治疗了 139 例患者。对主要终点（死亡和再次手术/介入）进行了风险因素分析。
随访率为 88%，平均随访时间为 16±7 年（中位数为 15 年[范围，1-48 年]），累计随访时间为 1739 患者-年。15%的患者接受了心房调转术，48%的患者接受了 Rastelli 手术，9%的患者接受了 Nikaidoh 手术，8%的患者接受了 REV 手术，20%的患者接受了动脉调转术。30 年时的生存率为 86%（范围，83%-90%），不同术式之间无差异。30 年时无初次手术的累积生存率为 17%（范围，12%-21%）。30 年时无 LVOTO 再次手术的生存率为 74%（范围，70%-78%）。9%的患者发生复发性 LVOTO，且危险因素为弥漫性室间隔下梗阻（比值比，9.8；P=.04）。除了右心室至肺动脉导管外，首次再次手术的 30 年无再发率为 60%（范围，59%-61%），预测因素为多发室间隔缺损（风险比，6；P=.03）和 Rastelli 手术（风险比，12；P=.03）。REV 手术的绝对再次手术率最低（12 年时为 16%）。
手术治疗大动脉转位合并室间隔缺损和 LVOTO 的长期生存率较好，不同术式之间无显著差异。自 21 世纪初以来，Nikaidoh 和 REV 手术的应用明显增多，其中 REV 手术的效果尤其好。Rastelli 手术后，以手术特异性和非特异性再次手术最常见。
