室间隔完整型肺动脉闭锁的右心室减压策略

目的总结室间隔完整型肺动脉闭锁(PA/IVS)右心室减压的经验与教训,反思右心室减压策略。方法回顾性分析2015年3月至2019年12月于我院行右心室减压手术的12例PA/IVS患儿的临床资料,其中男10例、女2例,手术时中位年龄5(1~627)d。对减压后肺动脉瓣跨瓣压差与三尖瓣Z值变化进行相关性分析。结果术后死亡1例,因术后并发循环分流导致顽固性低氧血症,家属拒绝再次手术。术后再干预2例(16.67%)。减压术后肺动脉瓣跨瓣压差为(31.95±21.75)mm Hg。肺动脉瓣轻度反流7例,中度反流2例,重度反流1例。机械通气中位时间30.50(6.00~270.50)h,监护室滞留时间(164.06±87.74)h。术后随访(354.82±331.37)d。末次随访时:三尖瓣Z值为1.32±0.71,肺动脉瓣跨瓣压差为41.75(21~146)mm Hg,经皮血氧饱和度为92.78%±3.73%。2例患儿分别于术后6个月和10个月再次予经皮球囊肺动脉瓣扩张术,免再干预率为81.8%。减压术后肺动脉瓣跨瓣压差与三尖瓣Z值的差值无明显相关性(r=–0.506,P=0.201)。结论PA/IVS患儿进行右心室减压时避免单纯追求充分减压而导致肺动脉瓣功能严重失功,增加无效循环分流风险,诱发顽固性低氧血症。实行分期减压可以保证初期手术安全性和有效性,降低术后死亡风险。

Right ventricular decompression for pulmonary atresia with intact ventricular septum

Objective To summarize the experience and lessons of right ventricular decompression in children with pulmonary atresia and intact ventricular septum(PA/IVS)and to reflect on the strategies of right ventricular decompression.Methods The clinical data of 12 children with PA/IVS who underwent right ventricular decompression in our hospital from March 2015 to December 2019 were reviewed retrospectively.There were 10 males and 2 females with a median age at the time of surgery was 5 d(range,1-627 d).Correlation analysis between the pulmonary valve transvalvular pressure gradient and changes in Z score of tricuspid valves after decompression was performed.Results One patient died of refractory hypoxemia due to circulatory shunt postoperatively and family members gave up treatment.There were 2(16.67%)patients received postoperative intervention.The pulmonary transvalvular gradient after decompression was 31.95±21.75 mm Hg.Mild pulmonary regurgitation was found in 7 patients,moderate in 2 patients,and massive in 1 patient.The median time of mechanical ventilation was 30.50 h(range,6.00-270.50 h),and the average duration of ICU stay was 164.06±87.74 h.The average postoperative follow-up time was 354.82±331.37 d.At the last follow-up,the average Z score of tricuspid valves was 1.32±0.71,the median pressure gradient between right ventricle and main pulmonary artery was 41.75 mm Hg(range,21-146 mm Hg)and the average percutaneous oxygen saturation was92.78%±3.73%.Two children underwent percutaneous balloon pulmonary valvoplasty at 6 and 10 months after surgery,respectively,with the rate of reintervention-free of 81.8%.There was no significant correlation between pulmonary transvalvular gradients after decompression and changes in Z score of tricuspid valves(r=–0.506,P=0.201).Conclusion For children with PA/IVS,the simple pursuit of adequate decompression during right ventricular decompression may lead to severe pulmonary dysfunction,increase the risk of ineffective circular shunt,and induce refractory hypoxemia.The staged decompression can ensure the safety and effectiveness for initial surgery and reduce the risk of postoperative death.