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Accuracy of Fetal Echocardiography in Defining Pulmonary Artery Anatomy and Source of Pulmonary Blood flow in Pulmonary Atresia with Ventricular Septal Defect (PA/VSD)

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Abstract

Precise delineation of central and branch pulmonary artery anatomy, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy in the fetal diagnosis of pulmonary atresia with ventricular septal defect is challenging but important to prenatal counseling and postnatal management. We aimed to evaluate the accuracy of fetal echocardiography to determine these anatomical nuances in pulmonary atresia with ventricular septal defect. This was a retrospective, single-institution, 10-year chart review of consecutive prenatal diagnosis of pulmonary atresia with ventricular septal defect for assessment of pulmonary artery, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy and comparison with postnatal imaging including echocardiography, cardiac catheterization, and computerized tomography angiography. Twenty-six fetuses were diagnosed with pulmonary atresia with ventricular septal defect during the review period and complete postnatal follow-up was available in 18, all confirming the basic prenatal diagnosis. Fetal echocardiography accurately predicted central and branch pulmonary artery anatomy in 16 (89%) [confluent in 14, discontinuous in 2], patent ductus arteriosus status in 15 (83%) [present in 10, absent in 5], and major aorto-pulmonary collateral arteries in 17 (94%) [present in 9, absent in 8]. Accuracy increased to 100% for pulmonary artery anatomy (16/16) and major aorto-pulmonary collateral artery (17/17) when excluding patients whose anatomy was reported as uncertain on fetal echocardiography. Fetal echocardiography can provide accurate anatomical details in the vast majority of fetuses with pulmonary atresia with ventricular septal defect. This allows for more anatomy-specific counseling, prognostication, and improved selection of postnatally available management options.

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Data Availability

De-identified participant data are available upon request from the corresponding author.

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No funding was received for conducting this study.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Seattle Children’s Hospital and University of Washington School of Medicine, 4800 Sand Point Way NE, Seattle, WA, 98105, USA

    Iman Naimi, Bhawna Arya, Jeffrey A. Conwell, Mark B. Lewin & Aarti H. Bhat

  2. Echocardiography Laboratory, Seattle Children’s Hospital, Seattle, WA, 98105, USA

    Michele Clouse

Authors
  1. Iman Naimi
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  2. Michele Clouse
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  3. Bhawna Arya
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  4. Jeffrey A. Conwell
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  5. Mark B. Lewin
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  6. Aarti H. Bhat
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Contributions

IN: Principal Investigator. Involved with the design of the study, constructing the research protocol, study and report review, IRB preparation and maintenance, preliminary interpretation of data, drafting original manuscript and revising versions based on feedback from co-authors, and submission of final approved version. MC: Performed data review and manuscript review. BA: Performed fetal cardiology study overview and manuscript review. JC: Performed fetal cardiology study overview and manuscript review. ML: Performed fetal cardiology study overview and manuscript review. AB: Corresponding author. Involved with the design of the study, crafting of the research protocol, study and report review, IRB preparation and maintenance, preliminary interpretation of data, drafting original manuscript, revising versions based on feedback from co-authors, and submission of final approved version.

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Correspondence to Aarti H. Bhat.

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Naimi, I., Clouse, M., Arya, B. et al. Accuracy of Fetal Echocardiography in Defining Pulmonary Artery Anatomy and Source of Pulmonary Blood flow in Pulmonary Atresia with Ventricular Septal Defect (PA/VSD). Pediatr Cardiol 42, 1049–1057 (2021). https://doi.org/10.1007/s00246-021-02579-0

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  • DOI: https://doi.org/10.1007/s00246-021-02579-0

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