中国循证儿科杂志 ›› 2021, Vol. 16 ›› Issue (3): 192-196.

• 论著 • 上一篇    下一篇

超声心动图对室间隔完整型肺动脉瓣闭锁和极重度肺动脉瓣狭窄胎儿的分型、评估及其出生后随访

邹鹏, 孙善权, 刘琴, 饶姣, 黄景思, 许伟滨, 李虹   

  1. 广东省妇幼保健院心脏中心  广州,511442


  • 收稿日期:2021-01-25 修回日期:2021-04-02 出版日期:2021-06-25 发布日期:2021-06-25
  • 通讯作者: 李虹

Echocardiographic classification, assessment and follow-up of therapy after birth in the fetus with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis

ZOU Peng, SUN Shanquan, LIU Qin, RAO Jiao, HUANG Jingsi, XU Weibin, LI Hong    

  1. Department of Cardiac Center,Guangdong Women and Children Hospital,Guangdong Children`s Hospital,Guangzhou 511442,China
  • Received:2021-01-25 Revised:2021-04-02 Online:2021-06-25 Published:2021-06-25
  • Contact: LI Hong

摘要: 背景:室间隔完整型肺动脉瓣闭锁(PA/IVS)和极重度肺动脉瓣狭窄(CPS)是一类较为少见的先天性心脏病,对PA/IVS和CPS胎儿进行详细的超声心动图评估并预测出生后的心脏情况,对于生育咨询非常重要。 目的:通过超声心动图对PA/IVS和CPS胎儿、新生儿、手术后病例进行分型与评估。 设计:病例系列报告。 方法:对超声心动图诊断PA/IVS和CPS的胎儿、新生儿、手术后病例的资料进行回顾性分析,比较胎儿期、新生儿期及治疗随访后的超声心动图特征。 主要结局指标:胎儿期、新生儿期及治疗随访后右心室的发育及肺动脉瓣开放的进展及相关性。 结果:100例PA/IVS和CPS胎儿中,Ⅰ、Ⅱ和Ⅲ型分别为69、19和12例。Ⅰ和Ⅱ型伴有中度至重度的三尖瓣关闭不全(TR),Ⅲ型未发现TR。Ⅲ型均为PA/IVS,其中有7例出现心室冠状动脉交通。在PA/IVS中,11例胎儿出现肺动脉的明显发育不良。生后接受治疗的23例新生儿中,15例为PA/IVS,8例为CPS,Ⅰ和Ⅱ型分别为21和2例。2例分别由胎儿期的中度和重度肺动脉狭窄(PS),至出生时进展为PA/IVS。新生儿期的右室长径与左室长径的比值(RV/LV长径比值)较胎儿期变小。胎儿与新生儿期的中至重度TR均有22例。在镶嵌治疗后,1例PA/IVS患儿在术后7 d死亡,22例门诊随访,三尖瓣Z值、TV/MV比值和RV/LV长径比值与术前相比显著性增加,手术前与随访后的中至重度TR由22例减少至1例。 结论:超声心动图可以对PA/IVS和CPS胎儿进行分型与评估,TR的程度轻而右室发育不良较重,胎儿期部分中至重度PS可至出生时进展为PA/IVS,胎儿右室发育不良程度呈加重变化。

关键词: 室间隔完整型肺动脉瓣闭锁, 极重度肺动脉瓣狭窄, 右心室发育不良, 三尖瓣关闭不全, 超声心动图

Abstract: Background: Pulmonary atresia with intact ventricular septum (PA/IVS) and critical pulmonary stenosis (CPS) are rare congenital heart diseases. It is vital for cardiologists and pregnant women to perform detailed echocardiography evaluation in order to predict postnatal cardiac conditions. Objective: This study is to classify and evaluate the fetal, neonatal and postoperative cases with PA/IVS or CPS. Design: Case series report. Methods: Medical records of the fetal, neonatal and postoperative cases diagnosed with PA/IVS or CPS by echocardiograghy were retrospectively analyzed, and the echocardiographic characteristics of fetal, neonatal cases and follow-up after treatment were compared. Main outcome measures: The progress and correlation of the growth of right ventricle and pulmonary valve opening during fetal, neonatal stages and follow-up after treatment. Results: Of the 100 fetuses with PA/IVS or CPS, the number of typeⅠ, type Ⅱ and type Ⅲ was 69, 19, and 12 respectively. Type Ⅰ and type Ⅱ had moderate to severe tricuspid regurgitation (TR), while type Ⅲ had no TR. Type Ⅲ cases were all PA/IVS, in which seven fetuses had ventriculocoronary connection. Eleven fetuses with PA/IVS showed obvious pulmonary aterary dysplasia. Of the 23 fetuses treated after birth, there were 15 for PA/IVS, 8 for CPS, 21 for typeⅠand 2 for type Ⅱ. Two patients developed from moderate and severe PS in the fetus to PA/IVS at birth, respectively. The ratio of right to left ventricular length (RV/LV) was lower in neonatal period than that in fetal period. Moderate-severe TR were 22 in both fetal and neonatal stages. After hybrid therapy, one patient with PA/IVS died 7 days after operation, and 22 patients were followed up in the out-patient setting. TV Z-score, TV/MV and RV/LV increased significantly compared with that of pre-operation, and moderate to severe TR decreased from 22 cases to 1 case after operation and follow-up. Conclusion: Fetuses with PA/IVS and CPS can be classified and evaluated by echocardiography. The degree of TR is mild, while right ventricular dysplasia is more serious. During the prenatal period, part of moderate to severe PS can progress to PA/IVS, and the degree of right ventricular dysplasia is aggravated.

Key words: Pulmonary atresia with intact ventricular septum, Critical pulmonary stenosis, Dysplasia of right ventricule, Tricuspid regurgitation, Echocardiography