Hypoplastic left heart syndrome with congenital complete atrioventricular block in one twin：a case report
10.3760/cma.j.cn131148-20250115-00021
- VernacularTitle:双胎之一胎儿左心发育不良综合征合并先天性完全性房室传导阻滞1例
- Author:
Xiaotian FU
1
;
Sheng ZHAO
1
;
Chen CHENG
1
;
Li ZHANG
1
;
Yuhan WU
1
;
Wenli HUANG
1
;
Fu LIU
1
Author Information
1. 湖北省妇幼保健院超声诊断科，武汉 430070
- Publication Type:Journal Article
- Keywords:
Echocardiography;
Hypoplastic left heart syndrome;
Congenital complete atrioventricular block;
Cerebroplacental ratio;
Cardiovascular overall performance
- From:
Chinese Journal of Ultrasonography
2025;34(7):633-636
- CountryChina
- Language:Chinese
-
Abstract:
Hypoplastic left heart syndrome is a congenital heart disease characterized by mitral and（or）aortic valve stenosis or atresia，leading to the hypoplasia of the left ventricle，ascending aorta and aortic arch.If not treated in time，most children die within a few weeks after birth.Congenital complete atrioventricular block is a rare congenital cardiac conduction disorder.When combined with severe cardiac malformations，the mortality rate is extremely high.In this case，a 26-year-old pregnant woman with a history of 3 pregnancies and 1 delivery，had a natural conception with monochorionic diamniotic twins. At 22 +5 weeks of gestation，it was first found that one of the twins had hypoplastic left heart syndrome with congenital complete atrioventricular block through fetal echocardiography detection.This situation was extremely rare，and there was a lack of clinical guidelines on how to manage the pregnancy and determine the time of delivery time.In this case，prenatal ultrasound was used to monitor and comprehensively evaluate fetal cardiac function through the cerebroplacental ratio（CPR）combined with the cardiovascular overall performance score（CVPS）.Pregnancy safety management was implemented，and a cesarean section was performed at an elective time.Another healthy fetus did not suffer any adverse effects. This case provides strong evidence for ensuring the safety of healthy fetus and mother in perinatal period.It is also confirmed that CPR and CVPS combined evaluation of fetal cardiac function and pregnancy outcome will be more accurate and comprehensive.This method is simple，accurate，non-invasive，and can be used as an effective method for clinical evaluation of fetal cardiac function.
