中文摘要：

目的比较未手术和手术的肺动脉闭锁合并室间隔缺损（pulmonary atresia with ventricular septal defect,PA/VSD）患者的病死率。方法纳入2004年7月至2014年7月确诊为PA/VSD的住院患者,排除合并其他严重影响血流动力学的复杂畸形患者。通过先天性心脏病随访平台数据库登记手术患者的临床资料和随访信息。对于未行手术的患者,通过医院信息系统（hospital information system,HIS）回顾性收集其临床信息,并于2014年11月统一进行电话随访,获得其生存信息。结果共纳入符合诊断标准的PA/VSD住院患者344例,其中5例于术前死亡,行手术治疗219例,未手术120例。手术患者的入院年龄、体质量低于未手术患者,差异有统计学意义（P〈0.05）;而未手术患者中IV型PA/VSD的比例显著高于手术患者,差异有统计学意义（P〈0.05）。行手术治疗的PA/VSD患者中,住院死亡24例,随访（2.50±2.22）年,期间死亡3例,累计病死率12.33%（27/219）。对51例未手术患者随访（4.16±2.14）年,病死率为41.18%（21/51）。结论手术的PA/VSD患者住院病死率较高,但术后早中期效果较好。早中期随访未手术患者的病死率显著高于手术治疗患者。PA/VSD患儿应尽早评估手术指征,个体化选择适宜的姑息或根治手术方式进行治疗。

英文摘要：

Objectives To compare the mortality in paitents with pulmonary atresia with ventricular septal defect（PA /VSD） with and without surgery. Methods In-hospital patients who were diagnosed as PA / VSD by ultrasonic cardiography（UCG） or computed tomography（CT） in single center from July 1, 2004 to July 1, 2014 were included.PA / VSD patients with concomitant complex congenital heart defects were excluded. Database was set up and exclusively used in collecting clinical and follow-up information on patients undergoing surgeries. For PA / VSD patients who did not receive surgery, clinical information was collected from the hospital information system（HIS） and unified retrospective follow-up was performed in November, 2014 to get their survival information. Results Overall, 344 in-hospital patients with PA / VSD were included. Among them, 5 died before surgery, 219 underwent surgeries and 120 did not. The age and weight at admission of the patients with surgery were significantly lower than those without（P〈0.05）, while the proportion of tyep IV PA / VSD was significantly higher（P〈0.05）. Of the 219 PA / VSD patients with surgery, 24 died before discharge and 3 died during the follow-up [（2.50 ±2.22） years ]. The overall mortality was 12.33%（27 / 219）.The average follow-up duration was（4.16±2.14） years for patients without surgery and the mortality rate was 41.18%（21 / 51）. Conclusions The postoperative in-hospital mortality rate of PA / VSD is still high, but the mid-term outcome is satisfactory. The mortality rate is significantly higher in PA / VSD patients without surgery. For neonates and infants with PA / VSD, evaluation on surgical indication and proper surgeries in accordance with individual situations should be performed as soon as possible once the diagnosis is clear.