动脉导管依赖的肺动脉闭锁合并室间隔缺损的外科治疗

Surgical treatment of patent ductus arteriosus dependent pulmonary atresia with ventricular septal defect

目的探讨动脉导管依赖的肺动脉闭锁合并室间隔缺损（PA／VSD）的外科治疗方法以及不同解剖分型的手术疗效的比较。方法回顾性分析广东省心血管病研究所2005年1月至2014年12月收治的99例PA／VSD患儿临床资料，男63例，女36例；年龄0．4～291．9个月，中位值8．2个月；体质量2．3—50．0kg，中位值6．5kg。PA／VSD的Castaneda分型I型61例，Ⅱ型38例；分期手术36例，根治手术63例。结果I型、Ⅱ型PA／VSD患儿体外循环时间、主动脉阻断时间、术前经皮血氧饱和度、手术时动脉导管的处理方式以及右心室流出道重建方式差异有统计学意义。分期手术和根治手术的体外循环时间、主动脉阻断时间、体外循环最低温度、手术前后经皮血氧饱和度和手术时动脉导管的处理方式差异有统计学意义。结论动脉导管依赖的PA／VSD患儿早期疗效满意，手术方式以及不同解剖分型的手术难度、早期疗效存在差异，需要个性化评估。

Objective To investigate surgical approaches and compare the clinical outcomes of patent ductus arteriosus （PDA） dependent pulmonary atresia with ventricular septal defect（PA/VSD） in different anatomical classifications. Methods To retrospectively analyze the clinical data of 99 PA/VSD cases from January 2005 to December 2014 in Guangdong Cardio- vascular Institute. Pulmonary blood flow was mainly supplied by PDA in these patients of this study, which fell into the category of type I or n of PA/VSD according to anatomical classifications. 36 females and 63 males were included in our study, with median age of 8.2 months（0.4 to 291.9 months） , and median weight of 6.5 kg（2.3 to 50.0 kg） , respectively. Of these patients, 61 cases and 38 cases were categorized as type Ⅰand type Ⅱ of PA/VSD, in which 36 patients and 63 patients underwent staged procedure or curative correction, respectively. Results There was statistical significance between type Ⅰ and type Ⅱ of PA/VSD patients in cardiopulmonary bypass duration, aortic cross-clamp time, preoperative oxygen saturation, surgical methods of PDA, and surgical approaches of right ventricular outflow tract（RVOT） reconstruction. In addition, there was also statistical significance between staged procedure and curative correction in cardiopulmonary bypass duration, aortic cross-clamp time, minimal temperature in cardiopulmonary bypass, preoperative and postoperative oxygen saturation, and surgical methods of PDA. Conclusion The short-term outcome of surgical treatment of PDA dependent PA/VSD is satisfactory. There are differences in surgical approaches and early effectiveness based on various different anatomical classifications, and therefore, individualized evaluation is required.