6例未行宫内干预的室间隔完整型肺动脉闭锁伴右心发育不良胎儿的生后处理及随访  

Postnatal management and follow-up of six fetuses affected by pulmonary atresia with intact ventricular septum and right ventricular hypoplasia without intrauterine intervention

作　　者：孙宏晓 罗刚[1] 泮思林[1] 王思宝 纪志娴[1] 陈涛涛[2] 王葵亮 Sun Hongxiao;Luo Gang;Pan Silin;Wang Sibao;Ji Zhixian;Chen Taotao;Wang Kuiliang(Heart Center,Women and Children's Hospital,Qingdao University,Qingdao 266034,China;Department of Ultrasound,Women and Children's Hospital,Qingdao University,Qingdao 266034,China)

机构地区：[1]青岛大学附属妇女儿童医院心脏中心,青岛266034 [2]青岛大学附属妇女儿童医院超声科,青岛266034

出　　处：《中华围产医学杂志》2022年第8期576-581,共6页Chinese Journal of Perinatal Medicine

基　　金：国家自然科学基金(81770316, 81970249);泰山学者工程(2018);青岛市科技惠民示范引导专项(20-3-4-47-nsh)。

摘　　要：目的总结室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum, PA/IVS)伴右心发育不良(right ventricular hypoplasia, RVH)胎儿的生后诊治及预后情况, 探讨现有胎儿肺动脉瓣球囊扩张术(fetal pulmonary valvuloplasty, FPV)指征的应用价值。方法前瞻性纳入2018年9月至2021年3月于青岛大学附属妇女儿童医院心脏中心就诊的、有FPV指征但因胎位或胎龄问题无法行FPV, 且接受产前产后一体化管理的胎儿PA/IVS伴RVH的单胎妊娠孕妇。总结产前胎儿超声心动图评价指标、生后诊治及随访结果。采用配对样本的Wilcoxon符号秩和检验进行统计学分析。结果研究期间就诊的胎儿PA/IVS孕妇35例, 有FPV指征者28例, 其中18例接受FPV, 10例因胎位或胎龄问题无法行FPV。这10例中的4例家属要求引产, 最终6例接受产前产后一体化管理者纳入分析。产前初诊超声心动图评估中位胎龄为28.9周(28.3~30.4周)。与产前初诊超声心动图评估相比, 6周后再次评估时胎儿右心室长径/左心室长径增大[0.8(0.6~0.9)与0.6(0.5~0.8)], 三尖瓣反流速度增快[4.7 m/s(3.2~5.1 m/s)与4.1 m/s(3.3~4.8 m/s)], 三尖瓣Z值[-0.8(-1.6~0.8)与0.4(-0.3~1.9)]和单心室预测评分[0.5分(0.0~2.0分)与2.0分(1.0~3.0分)]降低, 差异均有统计学意义(T值分别为-2.21、2.00、-2.20和2.00, P值均<0.05)。6例胎儿均活产出生, 中位出生胎龄38.9周(37.3~40.1周), 生后即刻转入心脏监护病房后的中位体重为3 425 g(3 100~4 160 g), 首次手术干预中位日龄为12.5 d(0.0~20 d)。中位随访时间为15个月(11.8~18.5个月)。产前初诊评估时4例胎儿单心室预测评分为1~2分, 2例为3分。随访期间无死亡, 4例患儿实现解剖层面双心室循环(产前初诊评估单心室预测评分1~3分), 1例实现临床双心室循环(单心室预测评分3分), 1例仍需进一步随访(单心室预测评分2分)。结论满足FPV指征但未行宫内干预的PA/IVS伴RVH胎儿生�Objective To explore the value of current indications for fetal pulmonary valvuloplasty(FPV)by summarizing the postnatal diagnosis,treatment,and prognosis of fetuses with pulmonary atresia with intact ventricular septum(PA/IVS)and right ventricular hypoplasia(RVH).Methods This prospective study was conducted at the Heart Center of Women and Children's Hospital,Qingdao University from September 2018 to March 2021,which included pregnant women who were(1)with fetal PA/IVS and RVH;(2)unable to receive FPV due to fetal position or gestational age despite the indications;(3)given integrated pre-and postnatal management.Prenatal fetal echocardiography assessment,postnatal diagnosis,treatment,and follow-up were summarized using Wilcoxon matched-pair signed-rank test.Results A total of 35 singleton pregnant women were diagnosed with fetal PA/IVS and RVH by ultrasonic cardiogram and admitted during the study period.Among the 28 fetuses meeting the FPV indications,18 underwent FPV,while the other 10 did not due to inappropriate fetal position or gestational age.After excluding four terminated pregnancies,the rest six cases were enrolled.The median gestational age at the initial prenatal fetal echocardiography diagnosis was 28.9 weeks(28.3-30.4 weeks).Compared with the initial evaluation,the fetal right ventricular to left ventricular length/diameter ratio[0.8(0.6-0.9)vs 0.6(0.5-0.8)]and tricuspid regurgitation velocity[4.7 m/s(3.2-5.1 m/s)vs 4.1 m/s(3.3-4.8 m/s)]were increased,while tricuspid valve Z value[-0.8(-1.6-0.8)vs 0.4(-0.3-1.9)]and single-ventricular predictive score[0.5(0.0-2.0)vs 2.0(1.0-3.0)]were decreased when re-evaluated six weeks later(T were-2.21,2.00,-2.20,and 2.00;all P<0.05).All of the six fetuses were born alive with a median gestational age of 38.9 weeks(37.3-40.1 weeks).The median weight was 3425 g(3100-4160)g after being transferred to cardiac intensive care unit.The median age was 12.5 d(0.0-20.0 d)at the first surgical intervention.The median follow-up duration was 15 months(11.8-18.5 months).At i

关 键 词：心脏缺损 先天性 肺动脉瓣闭锁 心室 先天畸形 胎儿心脏 气囊瓣膜成形术 随访研究 

分 类 号：R714.5[医药卫生—妇产科学]