Prenatal ultrasound features and causes of missed diagnosis of pulmonary artery sling concurrent with an aberrant right subclavian artery

doi:10.3877/cma.j.issn.1672-6448.2025.03.002

Abstract

Abstract:

Objective

To analyze the prenatal ultrasound features of fetal pulmonary artery sling（PAS） concurrent with an aberrant right subclavian artery （ARSA）， to assess its diagnostic accuracy， and to explore the potential causes of missed diagnosis.

Methods

The clinical data and prognosis of three fetuses with PAS concurrent with an ARSA diagnosed in Maternal and Child Health Hospital of Hubei Province from January 2021 to May 2024 were retrospectively analyzed. The characteristics of prenatal ultrasound and fetal echocardiography were analyzed and compared to autopsy and chromosomal findings.

Results

All the three cases had a poor prognosis. Despite continuing the pregnancy until delivery， one case died shortly after the delivery in an outside hospital. Two cases terminated the pregnancy. All three cases of PAS were complete， all with stenosis or atresia of the airway， including two cases of tracheal and bronchial stenosis and one case of high-grade airway obstruction syndrome （laryngeal atresia with posterior nasal aperture atresia）.The first positive findings of prenatal ultrasound in all the three cases were extracardiac malformations （2 cases of digestive malformations and 1 case of facial malformations） and excessive amniotic fluid； two cases were clearly diagnosed by fetal echocardiography， and one case had the double bubble sign， did not undergo fetal echocardiography during pregnancy， and achieved diagnosis after birth by CT. All the 3 cases were combined with other intra- and extra-cardiac malformations： 1 case with persistent left superior vena cava；2 with multiple digestive malformations （2 cases of duodenal stenosis or atresia and anal atresia； 1 case of esophageal atresia and trachea-esophageal fistula）； and 1 with multiple facial malformations （small lower jaw，low-set ears， and binaural malformations）. All the 3 cases were combined with polyhydramnios， and 1 case was combined with single umbilical artery. In 1 case， diagnostic whole-exome sequencing showed that the patient had approximately 2.37 Mb of duplicates detected in the region 22q11.1-q11.21.

Conclusion

PAS concurrent with an ARSA is frequently associated with various intra- and extracardiac malformations， as well as abnormalities in amniotic fluid， which are often the first notable findings on prenatal ultrasound. Fetal echocardiography plays a crucial role in establishing a definitive diagnosis.

Key words: Pulmonary artery sling, Aberrant right subclavian artery, Double vascular ring, Prenatal ultrasound, Fetal echocardiography

Jieming Li, Bin Yang, Xiaohong Yang, li Lu, Sheng Zhao, Kaili Guo, fan Yang, Hui Xie. Prenatal ultrasound features and causes of missed diagnosis of pulmonary artery sling concurrent with an aberrant right subclavian artery[J]. Chinese Journal of Medical Ultrasound (Electronic Edition), 2025, 22(03): 197-202.

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Figures/Tables 4

References 22

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项目	病例1	病例2	病例3
检查孕周（周）	22⁺⁶	31⁺⁶	23⁺⁵
心内畸形	-	PAS、ARSA	PAS、ARSA、PLSVC
心外畸形	十二指肠梗阻	小下颌、低位耳	高位气道- 喉闭锁、食道- 十二指肠- 肛门闭锁、气管食管瘘
胎儿附属物异常	羊水过多	羊水过多	羊水过多、单脐动脉
生后补充诊断	PAS，ARSA，气管及左、右主支气管狭窄，肛门闭锁，直肠会阴瘘	左耳道狭窄，左侧耳前瘘管，右耳畸形（耳廓部分缺失，杯状耳，无耳道，耳舟、耳甲艇消失）；副脾3 个	双侧后鼻孔骨性闭锁，距阑尾5 cm 处可见小肠重复；双肺单叶，副脾2 个
妊娠结局	出生，生后47 d 手术后夭折	终止妊娠	终止妊娠

项目	病例1	病例2	病例3
检查孕周（周）	22⁺⁶	31⁺⁶	23⁺⁵
心内畸形	-	PAS、ARSA	PAS、ARSA、PLSVC
心外畸形	十二指肠梗阻	小下颌、低位耳	高位气道- 喉闭锁、食道- 十二指肠- 肛门闭锁、气管食管瘘
胎儿附属物异常	羊水过多	羊水过多	羊水过多、单脐动脉
生后补充诊断	PAS，ARSA，气管及左、右主支气管狭窄，肛门闭锁，直肠会阴瘘	左耳道狭窄，左侧耳前瘘管，右耳畸形（耳廓部分缺失，杯状耳，无耳道，耳舟、耳甲艇消失）；副脾3 个	双侧后鼻孔骨性闭锁，距阑尾5 cm 处可见小肠重复；双肺单叶，副脾2 个
妊娠结局	出生，生后47 d 手术后夭折	终止妊娠	终止妊娠

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