室间隔完整型肺动脉闭锁分期治疗策略的疗效分析

Efficacy of staging surgery in pulmonary atresia with intact ventricular septum

目的评估一期动脉导管支架植入、二期外科手术、三期介入房间隔缺损封堵的分期治疗策略用于室间隔完整型肺动脉闭锁患者的可行性及疗效。 方法回顾性分析2009年1月至2017年12月在浙江大学医学院附属儿童医院心脏中心行动脉导管支架植入术治疗的室间隔完整型肺动脉闭锁患者19例的临床资料。男性11例，女性8例；年龄（13.8±7.7）d（范围：3～35 d），体重（3.4±0.5）kg（范围：2.8～4.1 kg）。Bull分型Ⅱ型13例，Ⅲ型6例。一期动脉导管支架植入后1、3、6个月门诊定期随访；二期外科手术后1、3、6个月及之后每年2次定期随访。手术前后血氧饱和度比较采用配对t检验。 结果19例患者均行动脉导管支架植入术，支架植入术后血氧饱和度较入院时明显提高[（86.0±3.0）%比（64.4±5.3）%，t＝16.353，P＝0.000]。随访过程中死亡1例，失访2例，1例因血氧饱和度下降行支架球囊成形术，术后血氧饱和度明显提升。12例完成二期手术治疗，术后死亡1例，其余均存活，其中双心室功能矫治2例，一个半心室功能矫治9例；2例已完成三期房间隔缺损封堵术。 结论一期动脉导管支架植入、二期外科手术、三期介入房间隔缺损封堵的分期手术治疗室间隔完整型肺动脉闭锁疗效良好，避免了多次外科矫治带来的创伤，可作为Bull分型Ⅱ、Ⅲ型及右心室重度发育不良的室间隔完整型肺动脉闭锁的重要治疗选择。

ObjectiveTo assess the feasibility and efficacy of a staged invasive treatment strategy for the treatment of pulmonary atresia with intact ventricular septum, in the first stage using a catheter-based stent placement, second-stage surgery, and three-stage atrial septal occlusion. MethodsTotally 19 children with pulmonary atresia with intact ventricular septum were enrolled at Department of Cardiovascular Surgery, the Children′s Hospital of Zhejiang University School of Medicine from January 2009 to December 2017, including 11 male and 8 female patients. The age was （13.8±7.7） days （ranging from 3 to 35 days）. The weight was （3.4±0.5） kg （ranging from 2.8 to 4.1 kg）. Among them, there were 13 cases of type Ⅱ and 6 cases of type Ⅲ. Regular follow-up visits for patients with stage Ⅰ arterial duct stenting after 1, 3, and 6 months; and routine follow-ups for 1, 3, and 6 months after stage Ⅱ surgery.The t test was used to compare the oxygen saturation between pre-operation and post-operation. ResultsAll 19 patients underwent arterial catheterization. The preoperative peripheral oxygen saturation was （64.4±5.3）%, while increased to （86.0±3.0）% after operation （t=16.353, P=0.000）. One patient died during follow-up and 2 patients lost follow-up. One patient received stent balloon dilatation due to oxygen saturation decrease.Twelve patients completed the second-stage surgery and 1 case died after surgery. The rest were alive, including 2 cases of double-ventricular correction and 9 cases of semi-ventricular treatment in one room; 2 cases had completed three-stage atrial septal occlusion. ConclusionsStage Ⅰ arterial duct stent, stage Ⅱ surgery, and stage Ⅲ atrial septal occlusion to treat pulmonary atresia with intact ventricular septum in children is feasible and effective. The method can be used as the important treatment direction for pulmonary atresia with intact ventricular septum.