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Quality of Life in Adults With Transposition of the Great Arteries With a Systemic Right or Left Ventricle

Ansari Ramandi, M.M. ; Hlebowicz, J. LU and van Melle, J.P. (2025) In Canadian Journal of Cardiology 41(12). p.2544-2553
Abstract
Background Advances in medical and surgical care have improved survival in patients with transposition of the great arteries (TGA), shifting focus toward quality of life (QoL). In this study we evaluate QoL in adults with TGA, including congenitally corrected TGA (ccTGA) and patients with dextro-TGA (d-TGA), by comparing patients with a systemic right ventricle (sRV) and systemic left ventricle (sLV), while identifying mediating factors. Methods This cross-sectional study, part of the APPROACH-IS II trial, included 798 adults with TGA from 42 centres worldwide. QoL was assessed using a linear analogue scale (0-100). Regression models identified variables associated with QoL, and mediation analysis assessed the effect of sRV on QoL. Results... (More)
Background Advances in medical and surgical care have improved survival in patients with transposition of the great arteries (TGA), shifting focus toward quality of life (QoL). In this study we evaluate QoL in adults with TGA, including congenitally corrected TGA (ccTGA) and patients with dextro-TGA (d-TGA), by comparing patients with a systemic right ventricle (sRV) and systemic left ventricle (sLV), while identifying mediating factors. Methods This cross-sectional study, part of the APPROACH-IS II trial, included 798 adults with TGA from 42 centres worldwide. QoL was assessed using a linear analogue scale (0-100). Regression models identified variables associated with QoL, and mediation analysis assessed the effect of sRV on QoL. Results Among participants (median age 34 years, 44.9% women), 504 (63.2%) had an sRV (ccTGA or d-TGA with atrial switch) and 294 (36.8%) had an sLV (ccTGA with double-switch operation or Rastelli and d-TGA with arterial switch or Rastelli). Patients with an sRV reported lower QoL (median 75, interquartile range 60-85) compared with those with an sLV (median 80, interquartile range 70-90; P < 0.001). The negative effect of sRV on QoL was mediated for 59% of patients by ventricular dysfunction (B = −2.37, 95% confidence interval −3.38 to −1.21; P < 0.001). Poorer QoL was independently associated with Asian race, employment status (job seeking, unemployed, or disabled), less social support, New York Heart Association functional class ≥ II, ventricular dysfunction, more interventional catheterizations, and depression/anxiety. Conclusions TGA patients with an sRV experience a lower QoL than those with an sLV, mediated mainly by ventricular dysfunction. Clinical Trial Registration NCT04902768. © 2025 The Authors. (Less)
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keywords
congenital, heart defects, mediation analysis, quality of life, systemic right ventricle, Adult, Arterial Switch Operation, Congenitally Corrected Transposition of the Great Arteries, Cross-Sectional Studies, Female, Heart Ventricles, Humans, Male, Middle Aged, Quality of Life, Transposition of Great Vessels, adult, anxiety, arterial switch operation, Article, Asian, catheterization, congenitally corrected transposition of the great arteries, controlled study, cross-sectional study, depression, disabled person, double switch operation, employment status, female, great vessels transposition, heart left ventricle, heart right ventricle, heart surgery, heart ventricle function, human, major clinical study, male, multicenter study, New York Heart Association class, Rastelli operation, social support, unemployment, clinical trial, complication, diagnostic imaging, heart ventricle, middle aged, pathophysiology, procedures, psychology, surgery
in
Canadian Journal of Cardiology
volume
41
issue
12
pages
10 pages
publisher
Elsevier
external identifiers
  • scopus:105024871012
  • pmid:40962221
ISSN
0828-282X
DOI
10.1016/j.cjca.2025.09.010
language
English
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yes
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93835c7a-44ce-4f22-98cf-55d22527f338
date added to LUP
2026-03-23 15:36:45
date last changed
2026-03-24 03:00:10
@article{93835c7a-44ce-4f22-98cf-55d22527f338,
  abstract     = {{Background Advances in medical and surgical care have improved survival in patients with transposition of the great arteries (TGA), shifting focus toward quality of life (QoL). In this study we evaluate QoL in adults with TGA, including congenitally corrected TGA (ccTGA) and patients with dextro-TGA (d-TGA), by comparing patients with a systemic right ventricle (sRV) and systemic left ventricle (sLV), while identifying mediating factors. Methods This cross-sectional study, part of the APPROACH-IS II trial, included 798 adults with TGA from 42 centres worldwide. QoL was assessed using a linear analogue scale (0-100). Regression models identified variables associated with QoL, and mediation analysis assessed the effect of sRV on QoL. Results Among participants (median age 34 years, 44.9% women), 504 (63.2%) had an sRV (ccTGA or d-TGA with atrial switch) and 294 (36.8%) had an sLV (ccTGA with double-switch operation or Rastelli and d-TGA with arterial switch or Rastelli). Patients with an sRV reported lower QoL (median 75, interquartile range 60-85) compared with those with an sLV (median 80, interquartile range 70-90; P &lt; 0.001). The negative effect of sRV on QoL was mediated for 59% of patients by ventricular dysfunction (B = −2.37, 95% confidence interval −3.38 to −1.21; P &lt; 0.001). Poorer QoL was independently associated with Asian race, employment status (job seeking, unemployed, or disabled), less social support, New York Heart Association functional class ≥ II, ventricular dysfunction, more interventional catheterizations, and depression/anxiety. Conclusions TGA patients with an sRV experience a lower QoL than those with an sLV, mediated mainly by ventricular dysfunction. Clinical Trial Registration NCT04902768. © 2025 The Authors.}},
  author       = {{Ansari Ramandi, M.M. and Hlebowicz, J. and van Melle, J.P.}},
  issn         = {{0828-282X}},
  keywords     = {{congenital; heart defects; mediation analysis; quality of life; systemic right ventricle; Adult; Arterial Switch Operation; Congenitally Corrected Transposition of the Great Arteries; Cross-Sectional Studies; Female; Heart Ventricles; Humans; Male; Middle Aged; Quality of Life; Transposition of Great Vessels; adult; anxiety; arterial switch operation; Article; Asian; catheterization; congenitally corrected transposition of the great arteries; controlled study; cross-sectional study; depression; disabled person; double switch operation; employment status; female; great vessels transposition; heart left ventricle; heart right ventricle; heart surgery; heart ventricle function; human; major clinical study; male; multicenter study; New York Heart Association class; Rastelli operation; social support; unemployment; clinical trial; complication; diagnostic imaging; heart ventricle; middle aged; pathophysiology; procedures; psychology; surgery}},
  language     = {{eng}},
  number       = {{12}},
  pages        = {{2544--2553}},
  publisher    = {{Elsevier}},
  series       = {{Canadian Journal of Cardiology}},
  title        = {{Quality of Life in Adults With Transposition of the Great Arteries With a Systemic Right or Left Ventricle}},
  url          = {{http://dx.doi.org/10.1016/j.cjca.2025.09.010}},
  doi          = {{10.1016/j.cjca.2025.09.010}},
  volume       = {{41}},
  year         = {{2025}},
}