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胎儿右位主动脉弓合并左肺动脉异常起源的产前诊断:病例报告及文献复习

Prenatal Diagnosis of Anomalous Origin of the Left Pulmonary Artery in Fetus with a Right-Sided Aortic Arch: Case Report and Review of the Literature.

作者信息

Szlang Laura, Chaoui Rabih, Opgen-Rhein Bernd, Cho Mi-Young, Henrich Wolfgang

机构信息

Department of Obstetrics, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Center for Prenatal Diagnosis and Human Genetics, Berlin, Germany.

出版信息

Fetal Diagn Ther. 2025;52(1):30-36. doi: 10.1159/000541432. Epub 2024 Sep 17.

Abstract

INTRODUCTION

Anomalous origin of the left pulmonary artery (AOLPA) is an exceptionally rare congenital malformation, requiring particular care to be detected during fetal echocardiography.

CASE PRESENTATION

A 30-year-old woman, gravida 1, para 0, was referred for a mid-trimester anomaly scan. The three-vessel tracheal view in fetal echocardiographic examination led to the prenatal detection of an AOLPA in the presence of a right-sided aortic arch. Additionally, a bilateral arterial duct and the ductal origin of the left pulmonary artery (LPA) were detected postnatally. Prenatal diagnosis enabled the scheduling of the delivery in a tertiary perinatal center, immediate postnatal treatment with prostaglandin E1 to avoid obstruction of the isolated LPA, as well as surgical repair of the anomaly.

CONCLUSION

The rareness of the disease led to only sporadically published cases of prenatal diagnosis of AOLPA. However, early detection makes prenatal diagnosis crucial regarding the infants' outcome. This case report underlines the importance of a meticulous examination of the bifurcation of the pulmonary trunk during fetal echocardiography.

INTRODUCTION

Anomalous origin of the left pulmonary artery (AOLPA) is an exceptionally rare congenital malformation, requiring particular care to be detected during fetal echocardiography.

CASE PRESENTATION

A 30-year-old woman, gravida 1, para 0, was referred for a mid-trimester anomaly scan. The three-vessel tracheal view in fetal echocardiographic examination led to the prenatal detection of an AOLPA in the presence of a right-sided aortic arch. Additionally, a bilateral arterial duct and the ductal origin of the left pulmonary artery (LPA) were detected postnatally. Prenatal diagnosis enabled the scheduling of the delivery in a tertiary perinatal center, immediate postnatal treatment with prostaglandin E1 to avoid obstruction of the isolated LPA, as well as surgical repair of the anomaly.

CONCLUSION

The rareness of the disease led to only sporadically published cases of prenatal diagnosis of AOLPA. However, early detection makes prenatal diagnosis crucial regarding the infants' outcome. This case report underlines the importance of a meticulous examination of the bifurcation of the pulmonary trunk during fetal echocardiography.

摘要

引言

左肺动脉异常起源(AOLPA)是一种极为罕见的先天性畸形,在胎儿超声心动图检查期间需要格外留意才能检测到。

病例介绍

一名30岁女性,孕1产0,因孕中期异常扫描前来就诊。胎儿超声心动图检查中的三血管气管视图导致在存在右侧主动脉弓的情况下产前检测到AOLPA。此外,出生后检测到双侧动脉导管以及左肺动脉(LPA)的导管起源。产前诊断使得能够在三级围产期中心安排分娩,产后立即使用前列腺素E1进行治疗以避免孤立的LPA梗阻,以及对该畸形进行手术修复。

结论

该疾病的罕见性导致仅有零星发表的AOLPA产前诊断病例。然而,早期检测使得产前诊断对于婴儿的预后至关重要。本病例报告强调了在胎儿超声心动图检查期间仔细检查肺动脉干分叉的重要性。

引言

左肺动脉异常起源(AOLPA)是一种极为罕见的先天性畸形,在胎儿超声心动图检查期间需要格外留意才能检测到。

病例介绍

一名30岁女性,孕1产0,因孕中期异常扫描前来就诊。胎儿超声心动图检查中的三血管气管视图导致在存在右侧主动脉弓的情况下产前检测到AOLPA。此外,出生后检测到双侧动脉导管以及左肺动脉(LPA)的导管起源。产前诊断使得能够在三级围产期中心安排分娩,产后立即使用前列腺素E1进行治疗以避免孤立的LPA梗阻,以及对该畸形进行手术修复。

结论

该疾病的罕见性导致仅有零星发表的AOLPA产前诊断病例。然而,早期检测使得产前诊断对于婴儿的预后至关重要。本病例报告强调了在胎儿超声心动图检查期间仔细检查肺动脉干分叉的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d1a4/11793089/78d412677688/fdt-2025-0052-0001-541432_F01.jpg

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