Contemporary surgical management of complete atrioventricular septal defect with tetralogy of Fallot in Japan
Contemporary surgical management of complete atrioventricular septal defect with tetralogy of Fallot in Japan
复制标题
日本完全性房室间隔缺损法洛四联症的现代外科治疗
DOI：
10.1007/s11748-022-01809-3
复制
发表时间：
2022
影响因子：
1.2
通讯作者：
Motomura Noboru
中科院分区：
文献类型：
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作者：
Sugimoto Ai;Tachimori Hisateru;Hirata Yasutaka;Sakamoto Kisaburo;Ota Noritaka;Shiraishi Shuichi;Tsuchida Masanori;Motomura Noboru
目的完全性房室间隔缺损合并法洛四联症是一种罕见而复杂的心脏病。本研究的目的是描述当代的管理方法，这种心脏病和outcomes.MethodsData获得了46个国内机构在日本心血管数据库（2011-2018）。入选患者的基本诊断为完全性房室间隔缺损伴法洛四联症，无其他复杂心脏病。主要结果是手术死亡率（30天或住院死亡率）。ResultsA共119例患者在本研究期间进行了首次手术，一个完整的房室间隔缺损与法洛四联症。在40例（34%）患者中进行了一期修复（一期修复组），在79例（66%）患者中进行了姑息手术，作为计划分期方法的一部分（分期组）。40家机构（87%）至少经历过一次分期修复。在研究期间，没有机构平均每年发生超过或等于2例病例。总体而言，发生了11例手术死亡（9.2%）。初次和分期组的手术死亡率相当（p= 0.5）。多因素分析显示，术前使用儿茶酚胺、重复姑息手术和急诊入院是手术死亡率的重要危险因素（比值比，95%可信区间：8.58，[0-0.11]; 12.65，[1.28-125.15]; 8.64，[1.87-39.32]）。结论完全性房室间隔缺损合并法洛四联症的分期手术是首选方法。这种复杂疾病的结局有利于完全性房室间隔缺损伴法洛四联症病例较少的中心患者。
ObjectivesComplete atrioventricular septal defect with tetralogy of Fallot is a rare and complex heart disease. This study aimed to describe contemporary management approaches for this heart disease and the outcomes.MethodsData were obtained from 46 domestic institutions in the Japan Cardiovascular Database (2011–2018). Patients with a fundamental diagnosis of complete atrioventricular septal defect with tetralogy of Fallot, without other complex heart diseases, were included. The primary outcome was operative mortality (30-day or in-hospital mortality).ResultsA total of 119 patients underwent initial surgery for a complete atrioventricular septal defect with tetralogy of Fallot during this study period. Primary repair was performed in 40 (34%) patients (primary repair group), and palliative procedure was performed in 79 (66%) patients as part of a planned staged approach (staged group). Forty institutions (87%) experienced at least one case of staged repair. No institution experienced more than or equal to two cases/year on average during the study period. Overall, 11 operative mortalities occurred (9.2%). Operative mortality rates in the primary and staged groups were comparable (p= 0.5). Preoperative catecholamine use, repeat palliative surgeries, and emergency admission were significant risk factors for operative mortality in multivariate analysis (odds ratio, 95% confidence interval: 8.58, [0–0.11]; 12.65, [1.28–125.15]; 8.64, [1.87–39.32, respectively]).ConclusionsStaged approach for complete atrioventricular septal defect with tetralogy of Fallot was the preferred option. The outcomes of this complex disease were favorable for patients in centers with low cases of complete atrioventricular septal defect with tetralogy of Fallot.