Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum

Objective: To summarize echocardiographic characteristics of the anatomy and hemodynamic and clinical outcomes in fetuses with isolated pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). Methods: This was a single-center retrospective study of fetuses with isolated...

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Main Authors: Chengcheng Pang, Yingyu Wang, Junjun Shen, Liuqing Yang, Yufen Li, Wei Pan
Format: Article
Language:English
Published: Elsevier 2024-07-01
Series:Pediatrics and Neonatology
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Online Access:http://www.sciencedirect.com/science/article/pii/S1875957223001766
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author Chengcheng Pang
Yingyu Wang
Junjun Shen
Liuqing Yang
Yufen Li
Wei Pan
author_facet Chengcheng Pang
Yingyu Wang
Junjun Shen
Liuqing Yang
Yufen Li
Wei Pan
author_sort Chengcheng Pang
collection DOAJ
description Objective: To summarize echocardiographic characteristics of the anatomy and hemodynamic and clinical outcomes in fetuses with isolated pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). Methods: This was a single-center retrospective study of fetuses with isolated PS or PA/IVS. Echocardiographic variables and clinical outcomes after delivery were evaluated and compared. Results: Between 2016 and 2021, 115 livebirths with isolated PS or PA/IVS were included. Proportion of fetuses with mild, moderate and critical PS and PA/IVS was 41.7 %, 18.3 %, 26.1 % and 13.9 %. Fetuses with more severe PS had worse anatomic and hemodynamic profiles. Specifically, the cardiothoracic ratio, pulmonary valve (PV) velocity, degree and velocity of tricuspid regurgitation increased as PS severity increased; and the pulmonary artery/aorta ratio, right ventricle/left ventricle long-axis (TV/MV) ratio, tricuspid valve/mitral valve annulus (TV/MV) ratio, and tricuspid valve inflow duration/cardiac cycle ratio decreased as PS severity increased (P <0.001 for all). PV velocity ≥2 m/s predicted PV pressure ≥40 mm Hg after delivery, with an AUC of 0.81; TV/MV ratio combined with RV/LV ratio predicted clinical outcomes, with an AUC of 0.88. Live births with more severe PS had higher mortality rate (mild 0 vs. moderate 0 vs. critical 11 % vs. PA-IVS 36 %) and lower rate of developing bi-ventricles (mild 100 % vs. moderate 95 % vs. critical 89 % vs. PA-IVS 36 %). Conclusion: Findings of this study help better understand the anatomy and hemodynamic and clinical outcomes in fetuses with isolated PS or PA/IVS, which could have implications for prenatal counseling and prediction of fetal outcome.
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spelling doaj-art-9ed1f17eeffb4b85af5a5f4220cc7d802025-08-20T03:03:42ZengElsevierPediatrics and Neonatology1875-95722024-07-0165432833510.1016/j.pedneo.2023.05.010Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septumChengcheng Pang0Yingyu Wang1Junjun Shen2Liuqing Yang3Yufen Li4Wei Pan5Department of Maternal-Fetal Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, China; Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, ChinaDepartment of Maternal-Fetal Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, China; Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, ChinaDepartment of Maternal-Fetal Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, China; Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, ChinaDepartment of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, ChinaDepartment of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, ChinaDepartment of Maternal-Fetal Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, China; Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, China; Corresponding author. Department of Maternal-Fetal Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Guangdong Province Key Laboratory of Structural Heart Disease, Southern Medical University, Guangzhou, 510080, Guangdong, ChinaObjective: To summarize echocardiographic characteristics of the anatomy and hemodynamic and clinical outcomes in fetuses with isolated pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). Methods: This was a single-center retrospective study of fetuses with isolated PS or PA/IVS. Echocardiographic variables and clinical outcomes after delivery were evaluated and compared. Results: Between 2016 and 2021, 115 livebirths with isolated PS or PA/IVS were included. Proportion of fetuses with mild, moderate and critical PS and PA/IVS was 41.7 %, 18.3 %, 26.1 % and 13.9 %. Fetuses with more severe PS had worse anatomic and hemodynamic profiles. Specifically, the cardiothoracic ratio, pulmonary valve (PV) velocity, degree and velocity of tricuspid regurgitation increased as PS severity increased; and the pulmonary artery/aorta ratio, right ventricle/left ventricle long-axis (TV/MV) ratio, tricuspid valve/mitral valve annulus (TV/MV) ratio, and tricuspid valve inflow duration/cardiac cycle ratio decreased as PS severity increased (P <0.001 for all). PV velocity ≥2 m/s predicted PV pressure ≥40 mm Hg after delivery, with an AUC of 0.81; TV/MV ratio combined with RV/LV ratio predicted clinical outcomes, with an AUC of 0.88. Live births with more severe PS had higher mortality rate (mild 0 vs. moderate 0 vs. critical 11 % vs. PA-IVS 36 %) and lower rate of developing bi-ventricles (mild 100 % vs. moderate 95 % vs. critical 89 % vs. PA-IVS 36 %). Conclusion: Findings of this study help better understand the anatomy and hemodynamic and clinical outcomes in fetuses with isolated PS or PA/IVS, which could have implications for prenatal counseling and prediction of fetal outcome.http://www.sciencedirect.com/science/article/pii/S1875957223001766congenital heart diseaseechocardiogramoutcomepulmonary stenosis
spellingShingle Chengcheng Pang
Yingyu Wang
Junjun Shen
Liuqing Yang
Yufen Li
Wei Pan
Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum
Pediatrics and Neonatology
congenital heart disease
echocardiogram
outcome
pulmonary stenosis
title Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum
title_full Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum
title_fullStr Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum
title_full_unstemmed Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum
title_short Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum
title_sort echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum
topic congenital heart disease
echocardiogram
outcome
pulmonary stenosis
url http://www.sciencedirect.com/science/article/pii/S1875957223001766
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