经皮心导管微导丝肺动脉瓣打孔并球囊扩张治疗新生儿室间隔完整型肺动脉瓣闭锁效果分析

Efficacy of transcatheter pulmonary valve perforation by micro-guidewire and balloon dilation in neonates with pulmonary atresia with intact ventricular septum

目的 探讨经皮心导管微导丝肺动脉瓣打孔并球囊扩张治疗新生儿室间隔完整型肺动脉瓣闭锁(PA-IVS)的疗效。方法 回顾分析2012年1月至2018年12月在上海交通大学医学院附属新华医院接受经心导管微导丝肺动脉瓣打孔并球囊扩张术的21例(男14例、女7例)PA-IVS新生儿的治疗及随访情况。所有患儿均采用经Simmons导管推送微导丝肺动脉瓣打孔技术,同时行肺动脉瓣球囊扩张术。术后1、3、6、12个月及之后每年随访超声心动图评估手术效果及右心室发育情况。组间比较采用t检验。结果 共纳入21例PA-IVS新生儿,其中产前诊断13例,中位手术年龄6日龄,体重(3.2±0.5)kg,球囊与瓣环比值1.19±0.12,术前经导管测量右心室收缩压(121±33)mmHg(1 mmHg=0.133 kPa),术后即刻压力为(47±13)mmHg。入组患儿随访中位时间30个月,所有入组患儿均实现双心室循环,无死亡及严重的并发症发生。随访1年及以上的患儿16例,末次随访肺动脉跨瓣压差(29±15)mmHg,右心室与左心室横径比值明显高于术前(0.86±0.10比0.73±0.13,t=-2.96,P=0.006),肺动脉瓣瓣环Z值明显高于术前(-1.41±0.89比-2.83±1.06,t=-3.65,P=0.001),三尖瓣瓣环Z值比同样明显高于术前(-0.52±0.29比-1.34±0.81,t=-3.55,P=0.001)。结论 经心导管微导丝肺动脉瓣打孔并球囊扩张术是PA-IVS新生儿期安全有效的首期治疗方案。

Objective To investigate the safety and efficacy of transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation in the treatment of neonatal pulmonary atresia with intact ventricular septum(PA-IVS).Methods The retrospective study included 21 neonates(14 males and 7 females)with PA-IVS who underwent transcatheter micro-guidewire pulmonary valve perforation and balloon dilation in Xinhua Hospital from January 2012 to December 2018.All patients underwent the pulmonary valve perforation by micro-guidewire through the Simmons catheter.During the follow-up period at 1,3,6,12 months postoperatively and annually thereafter,the operative efficacy and the development of the right ventricle(RV)were evaluated by echocardiography.Statistical analyses were performed using t test.Results A total of 21 neonates with PA-IVS were enrolled,and 13 cases were diagnosed prenatally.The median age of surgery was 6 days,the weight was(3.2±0.5)kg.The balloon/valve ratio was 1.19±0.12,and the RV pressure measured by catheter was(121±33)mmHg(1 mmHg=0.133 kPa).The immediate postoperative RV pressure was(47±13)mmHg.The median follow-up time was 30 months.All the cases enrolled achieved biventricular circulation without death and serious complications.According to the last follow-up data including 16 cases who were followed up for 1 year or longer,the pulmonary artery transvalvular pressure was(29±15)mmHg.The postoperation ratio of right to left ventricular transverse diameter was significantly higher than that before operation(0.86+0.10 vs.0.73+0.13,t=-2.96,P=0.006).Compared with preoperative data,the postoperation pulmonary valvular diameter Z-score was significantly higher(-1.41±0.89 vs.-2.83±1.06,t=-3.65,P=0.001)and the tricuspid valvular diameter Z-score was significantly higher(-0.52±0.29 vs.-1.34±0.81,t=-3.55,P=0.001).Conclusion Transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation is a safe and effective initial therapy for neonatal PA-IVS.