室间隔完整的严重肺动脉瓣狭窄或闭锁胎儿心脏介入治疗二例  

Prenatal interventional therapy in two cases with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum

作　　者：庞程程[1] 潘微[1] 张智伟[2] 周成斌[1] 李渝芬[2] 张旭[2] 韩凤珍[3] 孙云霞[4] 王晟[5] 庄建[6] Pang Chengcheng;Pan Wei;Zhang Zhiwei;Zhou Chengbin;Li Yufen;Zhang Xu;Han Fengzhen;Sun Yunxia;Wang Sheng;Zhuang Jian(Department of Maternal-Fetal Cardiology, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangdong Province Key Laboratory of Structural Heart Disease, Guangzhou 510080, China)

机构地区：[1]广东省心血管病研究所 广东省人民医院(广东省医学科学院) 广东省华南结构性心脏病重点实验室心脏母胎医学科,广州510080 [2]广东省心血管病研究所 广东省人民医院(广东省医学科学院)心儿科,广州510080 [3]广东省心血管病研究所 广东省人民医院(广东省医学科学院)产科,广州510080 [4]广东省心血管病研究所 广东省人民医院(广东省医学科学院)新生儿科,广州510080 [5]广东省心血管病研究所 广东省人民医院(广东省医学科学院)麻醉科,广州510080 [6]广东省心血管病研究所 广东省人民医院(广东省医学科学院)心外科,广州510080

出　　处：《中华儿科杂志》2018年第6期445-450,共6页Chinese Journal of Pediatrics

基　　金：广东省省级科技计划项目（2012806170027、2014A020213010、2015A020210062、2017A070701013）;广东省省属科研机构竞争性支持创新能力建设项目（20158070701008）

摘　　要：目的分析1例胎儿室间隔完整的肺动脉瓣闭锁（PA-IVS）和1例胎儿室间隔完整的危重肺动脉瓣狭窄（CPS-IVS）进行成功的宫内肺动脉瓣成形术（FPV）的诊治经过，初步探讨FPV的病例选择、技术方法及出生后结局。方法2016年9月及2017年2月就诊于广东省人民医院经胎儿超声心动图诊断为胎儿PA-IVS及CPS-IVS各1例。2例胎儿均存在明显的右心室发育不良、三尖瓣严重反流。采用超声心动图评估右心系统的发育及血流动力学指标，主要包括：胎儿三尖瓣/二尖瓣环、右心室/左心室长径、肺动脉瓣/主动脉瓣环、三尖瓣流入时间/心动周期、三尖瓣反流程度、动脉导管及静脉导管血流方向等。联合心脏母胎医学科、心儿科、心外科、产科、新生儿科、麻醉科等多学科会诊，评估病例的介入治疗适应证及手术时机。2例胎儿均于28周胎龄接受了经孕妇腹壁超声引导下的FPV。术后每2～4周返院复查1次，采用超声心动图观察肺动脉瓣的启闭，评估右心系统的发育及血流动力学指标的改善情况。结果2例胎儿FPV均取得技术上的成功。术后2～4周复查2例胎儿肺动脉瓣的开放均较前改善，术后5～8周复查例1胎儿肺动脉瓣出现明显再狭窄，例2无明显再狭窄。例1右心系统发育及血流动力学指标较前稍改善，术后5～8周三尖瓣/二尖瓣环、右心室/左心室长径、肺动脉瓣/主动脉瓣环、三尖瓣流入时间/心动周期分别由术前的0.56、0.42、0.85、0.26，增加至0.59、0.51、0.87、0.32，但动脉导管血流仍为反向。例2右心系统发育及血流动力学指标较前改善明显，术后5～8周三尖瓣/二尖瓣环、右心室/左心室长径、肺动脉瓣/主动脉瓣环、三尖瓣流入时间/心动周期分别由术前的0.70、0.63、0.91、0.35，增加至0.80、0.80、0.97、0.42，动脉导管血流变为双向。2例胎儿均活产。例1生后第8天接受肺动脉�ObjectivesTwo cases who underwent fetal pulmonary valvuloplasty （FPV） for pulmonary atresia with intact ventricular septum （PA-IVS） or critical pulmonary stenosis with intact ventricular septum （CPS-IVS） successfully were reported. The aim of the report was to explore the criteria for case selection, the technical essentials of FPV, and the postpartum outcome of the fetus.MethodsOne case with PA-IVS and the other with CPS-IVS were enrolled in September 2016 and February 2017 in Guangdong General Hospital, and both cases were diagnosed with severe right ventricular dysplasia and tricuspid regurgitation by fetal echocardiogram. Parameters of right ventricle development and hemodynamics from echocardiography included tricuspid/mitral annulus （TV/MV）, right ventricle/left ventricle long-axis （RV/LV）, pulmonary/aortic annulus （PV/AV）, tricuspid inflow duration/cardiac cycle, degree of tricuspid regurgitation （TR）, blood flow direction of arterial duct and ductus venosus. Multidisciplinary team including the maternal-fetal cardiology, pediatric cardiology, cardiac surgery, obstetrics, neonatology and anesthesiology was summoned to discuss the indications and timing of PFV. Two cases underwent ultrasound-guiding trans-abdominal PFV at the 28 weeks of gestational age. Echocardiography was performed to observe the opening and closing of the pulmonary valve, and to evaluate the development of right ventricle and improvement in hemodynamics every 2-4 weeks until delivery.ResultsFrom the technical perspective, pulmonary balloon valvuloplasty was successfully performed in these two cases. The opening of pulmonary valve improved in these two cases at 2-4 weeks after FPV. However, an obvious restenosis was detected in the first case at 5-8 weeks after FPV. In the first case, the echocardiography parameters including TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle increased from 0.56, 0.42, 0.85,0.26 to 0.59, 0.51, 0.87, 0.32 at 5-8 weeks after FPV, respectively. However, the direction

关 键 词：胎儿 超声心动描记术 肺动脉瓣狭窄 肺动脉瓣闭锁 气囊扩张术 

分 类 号：R725.4[医药卫生—儿科]