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Health-related quality of life outcomes for congenitally corrected transposition of the great arteries patients across the therapeutic spectrum

Published online by Cambridge University Press:  18 August 2025

Anish R. Katta Open the ORCID record for Anish R. Katta [Opens in a new window] ,
Shivni Patel ,
Miza S. Hammoud ,
Amol Pande ,
Andrew Toth ,
Samuel M. Hoenig ,
Yibei Zhu Open the ORCID record for Yibei Zhu [Opens in a new window] ,
Lin Chin ,
Rashed Mahboubi  and
Justin A. Robinson
...Show all authors
Anish R. Katta
Affiliation:
Case Western Reserve University School of Medicine, University Program, Cleveland, OH, Canada
Shivni Patel
Affiliation:
Case Western Reserve University School of Medicine, University Program, Cleveland, OH, Canada
Miza S. Hammoud
Affiliation:
Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, OH, Canada
Amol Pande
Affiliation:
Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, OH, Canada
Andrew Toth
Affiliation:
Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, OH, Canada
Samuel M. Hoenig
Affiliation:
Case Western Reserve University School of Medicine, University Program, Cleveland, OH, Canada
Yibei Zhu
Affiliation:
Case Western Reserve University School of Medicine, University Program, Cleveland, OH, Canada
Lin Chin
Affiliation:
Case Western Reserve University School of Medicine, University Program, Cleveland, OH, Canada
Rashed Mahboubi
Affiliation:
Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, OH, Canada
Justin A. Robinson
Affiliation:
Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, OH, Canada
Tara Karamlou*
Affiliation:
Department of Pediatric Cardiac Surgery, Akron Children’s Hospital, Akron, OH, Canada Division of Cardiothoracic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, Canada
*
Corresponding author: Tara Karamlou; Email: tkaramlou@akronchildrens.org
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Abstract

Objective:

Congenitally corrected transposition of the great arteries is a rare congenital cardiac condition with varied presentations, complicating treatment decisions. This study evaluates the impact of medical management, physiologic repair, and anatomic repair on health-related quality of life.

Methods:

A cross-sectional follow-up was conducted on 50 congenitally corrected transposition of the great arteries patients from a cohort of 240 at Cleveland Clinic (1995-2020). Health-related quality of life was assessed using MacNew and PROMIS-10 questionnaires. Echocardiographic data on systemic atrioventricular valvular regurgitation and systemic ventricular dysfunction were analysed. A time-varying coefficient model evaluated these factors’ impact on health-related quality of life.

Results:

Anatomic repair had significantly higher PROMIS-10 Physical T-scores compared to physiologic repair (median 50.9 vs 41.6, p = 0.04). MacNew Social scores were significantly higher for medical management compared to physiologic repair (median 6.8 vs 6.0, p = 0.02). Echocardiographic analysis revealed that systemic ventricular dysfunction had a stronger immediate impact on health-related quality of life. Systemic atrioventricular valve regurgitation showed a delayed negative effect, significant at 6-10 years after echo, although this effect gradually decreased over time. Anatomic repair patients had better systemic ventricular function (84.6% normal), and less systemic atrioventricular valve regurgitation (69.2% had none) compared to medical management and physiologic repair groups.

Conclusions:

Anatomic repair improves health-related quality of life in congenitally corrected transposition of the great arteries patients, with physiologic repair showing some gains over time. Health-related quality of life data should provide important guidance regarding treatment decisions, especially in well-balanced congenitally corrected transposition of the great arteries patients.

Keywords

congenitally corrected transposition of the great arterieshealth related quality of lifeCHDsurgery

Information

Type
Original Article
Information
Cardiology in the Young , Volume 35 , Issue 8 , August 2025 , pp. 1595 - 1601
Copyright
© The Author(s), 2025. Published by Cambridge University Press

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Footnotes

Anish Katta and Shivni Patel contributed equally to this work.

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