Abstract
Pulmonary atresia with an intact ventricular septum typically occurs in patients with concordant atrioventricular and ventriculoarterial connections. When it does occur in patients with discordant connections, it is most frequently seen in association with congenitally corrected transposition. We present a rare case of transposition of the great arteries with a ventricular septal defect (VSD) detected in fetal life which evolved throughout pregnancy resulting in the development of pulmonary atresia and severe restriction of the VSD.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Data Availability
The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.
References
Patel C, Spector M, Zahka K (2000) Congenitally corrected transposition with pulmonary atresia and intact ventricular septum. Cardiol Young 10(3):268–270
Dayan J, Peyvandi S, Moon-Grady A (2015) Pulmonary atresia with an intact ventricular septum in the setting of D-transposition of the great arteries with a hypoplastic left ventricle: fetal diagnosis. J Ultrasound Med 34(12):2313–2315
Gupta S, Anderson R, Kothari S (2013) Pulmonary atresia and intact ventricular septum with transposed arterial trunks. Cardiol Young 25(1):161–163
Hayes D, Arya B, Kleinman C (2012) Ventriculocoronary fistulae in a patient with transposition of the great arteries, hypoplastic left heart, and pulmonary atresia. Cardiol Young 23(1):111–113
Funding
This project received no funding.
Author information
Authors and Affiliations
Contributions
SD and OF: wrote the main manuscript text. CD, FD, and OF: prepared the figures. All authors reviewed and edited the manuscript.
Corresponding author
Ethics declarations
Conflict of interest
The authors have no financial conflicts of interests to disclose.
Ethical Approval
An exemption to the ethical approval process was provided by the hospital ethics committee.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Supplementary Information
Below is the link to the electronic supplementary material.
Supplementary file1—Video 1: Severe mitral regurgitation at 26 weeks’ gestation secondary to restriction of VSD and intimate relationship between mitral valve chordae and VSD (MP4 1940 kb)
Supplementary file2—Video 2: Shortened, thickened and echobright mitral valve chordae at 26 weeks’ gestation. Aorta arises from anterior right ventricle (MP4 16075 kb)
Rights and permissions
About this article
Cite this article
Duignan, S., Doddy, F., McMahon, C.J. et al. Severe Restriction of a VSD and Development of Pulmonary Atresia in a Patient with Transposition of the Great Arteries: Fetal Diagnosis. Pediatr Cardiol 43, 1926–1928 (2022). https://doi.org/10.1007/s00246-022-02944-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-022-02944-7