胎儿肺动脉瓣狭窄产前诊断、治疗与转归的临床研究

Clinical Study on Prenatal Diagnosis,Treatment and Outcome of Fetal Pulmo⁃nary Valve Stenosis

目的探讨肺动脉瓣狭窄(pulmonary valve stenosis,PS)在胎儿期的超声特点、出生后转归、手术干预手段及时机。方法选取2016年1月至2021年1月广东省人民医院胎儿心脏超声诊断为胎儿单纯PS并生后随诊的患儿79例,回顾性分析和总结其产前及生后的临床资料。结果胎儿PS按动脉导管血流方向(正向和逆向)分为2组,2组胎儿PS在肺动脉瓣血流速度、三尖瓣反流程度、主肺动脉与主动脉内径比值、三尖瓣瓣环与二尖瓣瓣环比值、右心室与左心室纵径比值、心胸面积比例比较,差异均有统计学意义(P<0.01)。手术患儿中75%(30/40)取得双心室结局,5%(2/40)取得一个半心室结局,20%(8/40)在B-T分流术后随访观察中,3.7%(3/18)死亡。结论胎儿心脏超声是产前诊断胎儿PS并且评估生后预后的重要手段。胎儿PS总体预后较好,由于部分患儿呈进展性改变,诊断胎儿PS后,尤其动脉导管血流方向改变、三尖瓣反流重度的胎儿应进入产前生后一体化诊疗体系,以改善患儿的预后,减少病死率。

Objectives To investigate the ultrasonographic characteristics,postoperative outcome,surgical intervention and timing for fetal pulmonary valve stenosis(PS).Methods Seventy-nine cases of fetal simple PS with postnatal followup diagnosed by fetal cardiac ultrasound in Guangdong Provincial People′s Hospital from January 2016 to January 2021 were selected to retrospectively analyze and summarize the prenatal and postnatal clinical data.Results According to artery catheter blood flow direction(forward and reverse),the 79 fetus with PS were divided into 2 groups.There were significant statistical differences of the pulmonary artery blood flow velocity,tricuspid value regurgitation degree,main pulmonary artery to aortic diameter ratio,tricuspid value annulus to mitral valve annulus ratio,right to left ventricular longitudinal diameter ratio,heart to chest area ratio between the two groups(P<0.01).Biventricular outcome was achieved in 75%(30/40)of the children who underwent surgery,and in 5%(2/40)of the children were with one-ventricle outcome,20%(8/40)of the children with B-T shunt were observed after follow-up,3.7%(3/18)died.Conclusions Fetal cardiac ultrasound is an important mean for prenatal diagnosis of fetal PS and evaluation of postnatal prognosis.Fetal isolated PS achieves good prognosis.Due to progressive changes in some cases,after diagnosis of fetal PS,especially for fetus with arterial flow direction change and severe tricuspid regurgitation,integrated prenatal and postnatal diagnosis and treatment system should be adopted to improve the prognosis and reduce mortality.