摘要目的：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2.3～50.0 kg，中位值6.5 kg。 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。

abstractsObjective 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 typeⅠorⅡof PA/VSD according to anatomical classifications.36 females and 63 males were included in our study, with me-dian 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 pa-tients, 61 cases and 38 cases were categorized as type Ⅰ and typeⅡ of PA/VSD, in which 36 patients and 63 patients under-went staged procedure or curative correction, respectively.Results There was statistical significance between typeⅠand typeⅡof 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 .