胎儿严重肺动脉瓣狭窄或闭锁的产前超声心动图诊断评估、右心室发育评估及宫内介入治疗的结局随访

目的探讨胎儿室间隔完整型严重肺动脉狭窄(critical pulmonary stenosis with intact ventricular septum,CPS/IVS)或室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的产前超声心动图诊断、右心室发育评估及宫内介入治疗的结局随访.方法回顾性分析广东省人民医院2016年9月至2018年12月6例产前超声心动图诊断为PA/IVS或CPS/IVS(1例诊断PA/IVS,5例为CPS/IVS)行胎儿肺动脉瓣球囊成形术(fetal pulmonary valvuloplasty,FPV)胎儿的产前超声心动图诊断、右心室发育评估及结局随访资料.结果6例胎儿诊断孕周为(26.48±2.15)周.6例胎儿术前三尖瓣环/二尖瓣环比值(tricuspid valve annulus/mitral valve annulus,TV/MV)分别为0.53、0.82、0.71、0.85、0.77、0.71,右心室纵径/左心室纵径比值(right ventricle length/left ventricle length,RV/LV)分别为0.42、0.63、0.52、0.61、0.75、0.61,三尖瓣流入时间/心动周期长度比值(tricuspid valve inflow duration/cardiac cycle length,TVID/CCL)比值分别为0.26、0.35、0.39、0.44、0.44、0.35,肺动脉瓣环/主动脉瓣环(pulmonary valve annulus/aortic annulus,PV/AV)比值分别为0.85、1.03、0.85、0.86、1.20、0.78.动态观察2周后,6例胎儿右心室各指标未见明显增长,于(29.45±1.19)周行FPV术,均取得技术性成功(100%),无宫内死亡,孕妇及胎儿无严重并发症.FPV术后,6例胎儿术后1~2周内TV/MV、RV/LV、TVID/CCL明显增长,术后2~6周增长趋于稳定,而PV/AV则在术后2~4周增长较明显.6例患儿分娩胎龄为(37.74±1.17)周,出生体质量(3.04±0.24)kg,于生后(15.33±7.31)d完成行一期手术,其中2例行外科手术,4例行FPV.1例于13个月于外院完成二次手术,完成心室修补,4例患儿完成双心室矫治,但1例患儿死于围术期的严重感染所致的多器官功能衰竭.存活的5例患儿血氧饱和度>95%,无右心衰竭症状.结论产前可通过超声评估CPS/IVS、PA/IVS胎儿右心室发育状况,筛选宫内介入治疗适应证,适时进行FPV,可促进右心室小梁部及三尖瓣发育,争取患儿生后实现双心室循环.

Prenatal echocardiographic diagnosis,assessment of right ventricle,outcome follow-up of intrauterine interventional therapy in fetuses with critical pulmonary stenosis or pulmonary atresia

XU Xin-yun;ZHANG Zhi-wei;ZHANG Xu;LI Yi-fan;PANG Cheng-cheng;SHEN Jun-jun;ZHOU Chengbin;ZHUANG Jian;WEN Shu-sheng;HAN Feng-zhen;SUN Yun-xia;PAN Wei(Department of Pediatric Cardiology,Guangdong Cardiovascular Institute,Guangdong Provincial People's Hospi.tal,Guangdong Academy of Medical Science,Guangzhou 510080,China)