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

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

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.

Objectives To explore the prenatal echocardiographic diagnosis,assessment of right ventricle,outcome follow-up of intrauterine interventional therapy in fetuses with critical pulmonary stenosis with intact ventricular septum(CPS/IVS)or pulmonary atresia with intact ventricular septum(PA/IVS).Methods Six fetuses with diagnoses of CPS/IVS(n=5)or PA/IVS(n=1)by prenatal echocardiography in Guangdong Provincial People's Hospital from September 2016 to December 2018,who received fetal pulmonary valvuloplasty(FPV),were selected to retrospectively analyze the characteristics of echocardiographic diagnoses,assessment of right heart and outcomes follow-up.Results Gestation weeks of the 6 fetuses was(26.48±2.15)weeks.The tricuspid valve annulus/mitral valve annulus(TV/MV)ratios were 0.53,0.82,0.71,0.85,0.77 and 0.71,respectively.Right ventricle length/left ventricle length(RV/LV)ratios were 0.42,0.63,0.52,0.61,0.75 and 0.61,respectively.Tricuspid valve inflow duration/cardiac cycle length(TVID/CCL)ratios were 0.26,0.35,0.39,0.44,0.44 and 0.35,while pulmonary valve annulus/aortic valve annulus(PV/AV)ratios were 0.85,1.03,0.85,0.86,1.20 and 0.78,respectively.With two-week follow-up observations,the indicators of right ventricle in the fetuses showed no growth.All the cases were performed with FPV at the mean gestation week of(29.45±1.19)weeks and were technically successful,with no intrauterine death and severe complications in the mothers and fetuses.The TV/MV,RV/LV and TVID/CCL ratios increased obviously one to two weeks after FPV and trend to be stable in the later trimester,but PV/AV ratio increased two to four weeks after the operation.Six cases bored at(37.74±1.17)weeks,with average birthweight of(3.04±0.24)kg,and received one-stage operations in our department at(15.33±7.31)d.Two cases received surgical operations,and 4 cases underwent percutaneous balloon pulmonary valvuloplasty.One patient completed one and a half ventricular circulation in other department with bidirectional Glenn operation.Four cases had achieved biventricular circulation,but 1 patient died of multiple organ failure caused by severe affection in the perioperative period.Five cases survived had more than 95%oxygen saturation and no right ventricular dysfunction.Conclusions The prenatal echocardiographic diagnoses and accurate evaluation of right ventricle in fetus with CPS/IVS or PA/IVS could select appropriate participants of FPV and timely intervention,which could improve right heart growth,the function of tricuspid valve and the option of biventricular circulation.