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Transposition Physiology in the Setting of Concordant Ventriculo-arterial Connections
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  • Ujjwal Chowdhury,
  • Robert Anderson,
  • Diane E. Spicer,
  • Niwin George,
  • Lakshmi Sankhyan,
  • Niraj Pandey,
  • Shikha Goja,
  • Balaji Chandhirasekar
Ujjwal Chowdhury
All India Institute of Medical Sciences Cardio-Thoracic Sciences Centre
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Robert Anderson
Newcastle University School of Biomedical Nutritional and Sport Science
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Diane E. Spicer
Johns Hopkins All Children's Hospital Heart Institute
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Niwin George
All India Institute of Medical Sciences Cardio-Thoracic Sciences Centre
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Lakshmi Sankhyan
All India Institute of Medical Sciences Bilaspur Himachal Pradesh
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Niraj Pandey
All India Institute of Medical Sciences Cardio-Thoracic Sciences Centre
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Shikha Goja
All India Institute of Medical Sciences Cardio-Thoracic Sciences Centre
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Balaji Chandhirasekar
All India Institute of Medical Sciences Cardio-Thoracic Sciences Centre
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Abstract

Background and Aim: To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re-interventions, for the rare instances of transposition physiology with concordant ventriculo-arterial connections. Methods: We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo-arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Results: Among reported cases, just over two-thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four-fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one-fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multi-staged. Overall mortality was 25%, with one-fifth of patients requiring pacemakers for surgically-induced heart block. The majority of survivors were in good functional state. Conclusions: The flow patterns produced by discordant atrioventricular and concordant ventriculo-arterial connections remain an important, albeit rare, indication for atrial redirection. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long-term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits.
21 Mar 2022Submitted to Journal of Cardiac Surgery
21 Mar 2022Submission Checks Completed
21 Mar 2022Assigned to Editor
31 Mar 2022Reviewer(s) Assigned
19 Apr 2022Review(s) Completed, Editorial Evaluation Pending
19 Apr 2022Editorial Decision: Revise Minor
25 Apr 20221st Revision Received
25 Apr 2022Submission Checks Completed
25 Apr 2022Assigned to Editor
09 May 2022Review(s) Completed, Editorial Evaluation Pending
09 May 2022Editorial Decision: Accept