Department of Cardiovascular Surgery, Kobe Children's Hospital, Kobe, Hyogo, Japan.
Department of Cardiovascular Surgery, Kobe Children's Hospital, Kobe, Hyogo, Japan.
Ann Thorac Surg. 2014 May;97(5):1652-8. doi: 10.1016/j.athoracsur.2013.11.022. Epub 2014 Feb 6.
Unilateral pulmonary agenesis or aplasia (UPA), a rare developmental defect of the lung, is sometimes associated with congenital heart and tracheal diseases. The purpose of this study was to assess our experience of pediatric cardiothoracic surgery in UPA patients.
Cardiothoracic surgery for congenital heart defect or tracheal stenosis performed between 1981 and 2010 in 8 UPA patients (agenesis in 5 and aplasia in 3) was reviewed retrospectively. Associated cardiac anomalies included ventricular septal defect, double outlet right ventricle with pulmonary atresia, total anomalous pulmonary venous connection, and interrupted aortic arch complex.
For 7 patients with right UPA and 1 patient with left UPA, 12 cardiothoracic operations were performed, including 8 cardiac procedures in 4 patients and 4 tracheal procedures in 4 patients. Cardiac palliative repairs included Blalock-Park anastomosis, systemic-to-pulmonary artery shunt, and pulmonary artery banding. Cardiac definitive repairs included ventricular septal defect closure, subaortic membrane resection, modified Konno procedure, total anomalous pulmonary venous connection repair, and Rastelli-type operation. Tracheal repairs were costal cartilage tracheoplasty and slide tracheoplasty. The median age at surgery was 8 months and median body weight was 6.2 kg; the median operative time was 6.5 hours. There were 3 hospital deaths and 1 late death, with the 1-year mortality rate of 25%. Cardiopulmonary bypass-induced acute lung injury has occurred in 3 cases, 2 of which required extracorporeal membrane oxygenation support. Younger age of less than 1 month and prolonged cardiopulmonary bypass time of more than 200 minutes were related to operative risk factors for hospital mortality and morbidity.
Most of the pediatric cardiothoracic operations in UPA patients were successfully performed through an optimal surgical approach and procedure, but they still presented surgical risks of high mortality and morbidity. Perioperative management of UPA patients should be provided with a precise understanding of anatomic configuration and a careful consideration of underlying risk factors.
单侧肺发育不全或发育不良（UPA）是一种罕见的肺部发育缺陷，有时与先天性心脏和气管疾病有关。本研究旨在评估我们在 UPA 患者中行心胸外科手术的经验。
回顾性分析 1981 年至 2010 年间对 8 例 UPA 患者（5 例发育不全，3 例发育不良）行先天性心脏病或气管狭窄心胸外科手术的治疗经验。合并的心脏畸形包括室间隔缺损、右心室双出口伴肺动脉瓣闭锁、完全性肺静脉异位连接和主动脉弓中断。
7 例右侧 UPA 和 1 例左侧 UPA 患者共进行了 12 次心胸外科手术，其中 4 例患者进行了 8 次心脏手术，4 例患者进行了 4 次气管手术。心脏姑息性修复术包括 Blalock-Park 吻合术、体肺分流术和肺动脉缩窄术。心脏根治性修复术包括室间隔缺损修补术、主动脉瓣下膜切除术、改良 Konno 手术、完全性肺静脉异位连接修补术和 Rastelli 手术。气管修复术为肋软骨气管成形术和滑动气管成形术。手术中位年龄为 8 个月，中位体重为 6.2kg；中位手术时间为 6.5 小时。共有 3 例院内死亡和 1 例晚期死亡，1 年死亡率为 25%。3 例发生体外循环引起的急性肺损伤，其中 2 例需要体外膜氧合支持。年龄小于 1 个月和体外循环时间超过 200 分钟是与院内死亡率和发病率相关的手术危险因素。
大多数 UPA 患者的心胸外科手术通过优化的手术方法和步骤成功进行，但仍存在高死亡率和发病率的手术风险。对 UPA 患者应在围手术期进行精确的解剖结构了解，并仔细考虑潜在的危险因素。
