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CC BY-NC-ND 4.0 · Indian J Radiol Imaging
DOI: 10.1055/s-0044-1787684
Case Report

Total Anomalous Pulmonary Venous Connection with Rare Direct Right Atrial Drainage and Unprecedented Array of Coexistent Multisystem Variations

Arun Sharma 
1   Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India
,
Dollphy Garg   *
1   Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India
,
Shivali Arya
1   Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India
,
Sanjeev Hanumantacharya Naganur
2   Department of Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
,
Manphool Singhal 
1   Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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Abstract

Total anomalous pulmonary venous connection (TAPVC) is anomalous drainage of all pulmonary veins into systemic circulation. The intracardiac type typically entails the drainage of all the pulmonary veins into the right atrium, via the coronary sinus. The connection of the pulmonary veins directly into the right atrium is exceptionally rare and has been primarily reported with right atrial isomerism. Herein, we presented a remarkable case of TAPVC in a 10-year-old male child, distinguished by an unconventional drainage of all the pulmonary veins directly into the right atrium, with normal coronary sinus and absent right atrial isomerism. Intriguingly, computed tomography imaging revealed a combination of incredibly rare coexistent pulmonary, vascular, and skeletal anomalies. These anomalies included absence of pulmonary fissures in the right lung, presence of left circumflex aortic arch with bovine branching pattern, bilateral cervical ribs, and C7 vertebral fusion anomalies. To our knowledge, this unique combination of coexistent anomalies has not been previously reported in scientific literature in the background of rare drainage pattern of TAPVC.

Keywords

TAPVC - cardiac TAPVC - MDCT - pulmonary venous drainage - circumflex aortic arch

* Contributed equally with the first author and shares the first authorship.




Publication History

Article published online:
18 June 2024

© 2024. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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