Prenatal diagnosis and postnatal verification in fetuses with total anomalous pulmonary venous connection

Xiaoying Xue; Qiumei Wu; Mingtao Xiong; Wen Ling; Shan Guo; Hong Ma; Biying Huang; Min Liu; Xiuqing Qiu; Zongjie Weng

doi:10.3389/fped.2023.1206032

Prenatal diagnosis and postnatal verification in fetuses with total anomalous pulmonary venous connection

Front Pediatr. 2023 Jun 7:11:1206032. doi: 10.3389/fped.2023.1206032. eCollection 2023.

Authors

Xiaoying Xue^#¹, Qiumei Wu^#¹, Mingtao Xiong^#², Wen Ling¹, Shan Guo¹, Hong Ma³, Biying Huang¹, Min Liu¹, Xiuqing Qiu⁴, Zongjie Weng¹

Affiliations

¹ Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
² Department of Ultrasound, International Peace Maternity & Child Health Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
³ Department of Pathology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
⁴ Department of Obstetrics & Gynecology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.

^# Contributed equally.

Abstract

Objective: To systematically verify the accuracy of a four-step prenatal ultrasonography in diagnosing fetal total anomalous pulmonary venous connection (TAPVC).

Methods: A total of 62 TAPVC fetuses received prenatal ultrasonography and were confirmed by postnatal echocardiography, surgery, or postabortion autopsy. The suspected TAPVC fetuses were further screened by a four-step prenatal ultrasonography for TAPVC classification, pulmonary venous obstruction, and the associated malformations, and followed postpartum. The sonographic features, clinical data, and prognosis of the TAPVC fetuses were retrospectively analyzed.

Results: Of the 62 TAPVC fetuses, supracardiac TAPVC was found in 20 cases, intracardiac TAPVC in 12, infracardiac TAPVC in 21, and mixed TAPVC in 9. A total of 30 cases with right atrium isomerism were correctly diagnosed. Of the 11 cases with other intracardiac and extracardiac malformations, 1 case was missed to be diagnosed. Of the 21 isolated TAPVC cases, 6 were missed prenatally and 1 case was prenatally diagnosed as intracardiac and postnatally proved to be mixed (intracardiac type + supracardiac type) by echocardiography. Of the 13 TAPVC live births, 4 infants died in the neonatal period without operation. Of the nine infants undergoing the operation, five recuperated and survived; one survived but had complications with superior vena cava obstruction and collateral circulation formation, and three died postoperatively.

Conclusion: The four-step prenatal ultrasound procedure can comprehensively and systematically evaluate fetal TAPVC, detailing the classification, potential obstruction, and associated malformations. It provides substantial support for subsequent prenatal counseling and neonatal assessment. The retrospective analysis also reveals that isolated TAPVC is more prone to be missed in diagnosis.

Keywords: four-step approach; postnatal verification; prenatal diagnosis; total anomalous pulmonary venous connection; ultrasound.

Grants and funding

This publication was supported by Joint Funds for the Innovation of Science and Technology, Fujian province (2020Y9156); this publication was also supported by Fujian Natural Science Foundation (2021J01420).