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

Mohammad Mostafa Ansari Ramandi; Liesbet Van Bulck; Daan C H Ceelen; Adriaan A Voors; Eva Goossens; Adrienne H Kovacs; Koen Luyckx; Alexander Van De Bruaene; Harald Gabriel; Birgitte Lykkeberg; Corina Thomet; Michèle de Hosson; Magalie Ladouceur; Arwa Saidi; Diamantis Kosmidis; Maria Emília Areias; Joana Miranda; Camilla Sandberg; Zacharias Mandalenakis; Louise Coats; Pascal Amedro; Paul Khairy; Anne Marie Valente; Bengt Johansson; Anna Kaneva; Brith Andresen; Christina Christersson; Fernando Baraona Reyes; Joanna Hlebowicz; Junko Enomoto; Yuli Y Kim; Samuel Menahem; Hsiao-Ling Yang; Ju Ryoung Moon; Charlene Bredy; André Schmidt; Edward Callus; John Jairo Araujo; Andrew Constantine; Ali Zaidi; Judith Bouchardy; Susan M Jameson; Shelby Kutty; Lidija B McGrath; Ming Chern Leong; Lucia Ortiz; Fatma Demir Korkmaz; Maryanne Caruana; Mohamed Leye; Philip Moons; APPROACH-IS II consortium

doi:10.1016/j.cjca.2025.09.010

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

Mohammad Mostafa Ansari Ramandi, Liesbet Van Bulck, Daan C H Ceelen, Adriaan A Voors, Eva Goossens, Adrienne H Kovacs, Koen Luyckx, Alexander Van De Bruaene, Harald Gabriel, Birgitte Lykkeberg, Corina Thomet, Michèle de Hosson, Magalie Ladouceur, Arwa Saidi, Diamantis Kosmidis, Maria Emília Areias, Joana Miranda, Camilla Sandberg, Zacharias Mandalenakis, Louise CoatsPascal Amedro, Paul Khairy, Anne Marie Valente, Bengt Johansson, Anna Kaneva, Brith Andresen, Christina Christersson, Fernando Baraona Reyes, Joanna Hlebowicz, Junko Enomoto, Yuli Y Kim, Samuel Menahem, Hsiao-Ling Yang, Ju Ryoung Moon, Charlene Bredy, André Schmidt, Edward Callus, John Jairo Araujo, Andrew Constantine, Ali Zaidi, Judith Bouchardy, Susan M Jameson, Shelby Kutty, Lidija B McGrath, Ming Chern Leong, Lucia Ortiz, Fatma Demir Korkmaz, Maryanne Caruana, Mohamed Leye, Philip Moons, APPROACH-IS II consortium

Afdeling for Hjertesygdomme HJE

1 Citationer (Scopus)

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 < 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 TRIALS REGISTRATION: NCT04902768.

Originalsprog	Engelsk
Tidsskrift	The Canadian journal of cardiology
Vol/bind	41
Udgave nummer	12
Sider (fra-til)	2544-2553
Antal sider	10
ISSN	0828-282X
DOI	https://doi.org/10.1016/j.cjca.2025.09.010
Status	Udgivet - dec. 2025

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10.1016/j.cjca.2025.09.010Licens: CC BY

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Ansari Ramandi, M. M., Van Bulck, L., Ceelen, D. C. H., Voors, A. A., Goossens, E., Kovacs, A. H., Luyckx, K., Van De Bruaene, A., Gabriel, H., Lykkeberg, B., Thomet, C., de Hosson, M., Ladouceur, M., Saidi, A., Kosmidis, D., Areias, M. E., Miranda, J., Sandberg, C., Mandalenakis, Z., ... APPROACH-IS II consortium (2025). Quality of Life in Adults with Transposition of the Great Arteries with a Systemic Right or Left Ventricle. The Canadian journal of cardiology, 41(12), 2544-2553. https://doi.org/10.1016/j.cjca.2025.09.010

Ansari Ramandi, MM, Van Bulck, L, Ceelen, DCH, Voors, AA, Goossens, E, Kovacs, AH, Luyckx, K, Van De Bruaene, A, Gabriel, H, Lykkeberg, B, Thomet, C, de Hosson, M, Ladouceur, M, Saidi, A, Kosmidis, D, Areias, ME, Miranda, J, Sandberg, C, Mandalenakis, Z, Coats, L, Amedro, P, Khairy, P, Valente, AM, Johansson, B, Kaneva, A, Andresen, B, Christersson, C, Reyes, FB, Hlebowicz, J, Enomoto, J, Kim, YY, Menahem, S, Yang, H-L, Moon, JR, Bredy, C, Schmidt, A, Callus, E, Araujo, JJ, Constantine, A, Zaidi, A, Bouchardy, J, Jameson, SM, Kutty, S, McGrath, LB, Leong, MC, Ortiz, L, Korkmaz, FD, Caruana, M, Leye, M, Moons, P & APPROACH-IS II consortium 2025, 'Quality of Life in Adults with Transposition of the Great Arteries with a Systemic Right or Left Ventricle', The Canadian journal of cardiology, bind 41, nr. 12, s. 2544-2553. https://doi.org/10.1016/j.cjca.2025.09.010

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title = "Quality of Life in Adults with Transposition of the Great Arteries with a Systemic Right or Left Ventricle",

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 < 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 TRIALS REGISTRATION: NCT04902768.",

keywords = "Adult, Arterial Switch Operation/methods, Congenitally Corrected Transposition of the Great Arteries/psychology, Cross-Sectional Studies, Female, Heart Ventricles/physiopathology, Humans, Male, Middle Aged, Quality of Life, Transposition of Great Vessels/psychology, heart defects, congenital, mediation analysis, systemic right ventricle, quality of life",

author = "{Ansari Ramandi}, {Mohammad Mostafa} and {Van Bulck}, Liesbet and Ceelen, {Daan C H} and Voors, {Adriaan A} and Eva Goossens and Kovacs, {Adrienne H} and Koen Luyckx and {Van De Bruaene}, Alexander and Harald Gabriel and Birgitte Lykkeberg and Corina Thomet and {de Hosson}, Mich{\`e}le and Magalie Ladouceur and Arwa Saidi and Diamantis Kosmidis and Areias, {Maria Em{\'i}lia} and Joana Miranda and Camilla Sandberg and Zacharias Mandalenakis and Louise Coats and Pascal Amedro and Paul Khairy and Valente, {Anne Marie} and Bengt Johansson and Anna Kaneva and Brith Andresen and Christina Christersson and Reyes, {Fernando Baraona} and Joanna Hlebowicz and Junko Enomoto and Kim, {Yuli Y} and Samuel Menahem and Hsiao-Ling Yang and Moon, {Ju Ryoung} and Charlene Bredy and Andr{\'e} Schmidt and Edward Callus and Araujo, {John Jairo} and Andrew Constantine and Ali Zaidi and Judith Bouchardy and Jameson, {Susan M} and Shelby Kutty and McGrath, {Lidija B} and Leong, {Ming Chern} and Lucia Ortiz and Korkmaz, {Fatma Demir} and Maryanne Caruana and Mohamed Leye and Philip Moons and {APPROACH-IS II consortium}",

note = "Copyright {\textcopyright} 2025. Published by Elsevier Inc.",

year = "2025",

month = dec,

doi = "10.1016/j.cjca.2025.09.010",

language = "English",

volume = "41",

pages = "2544--2553",

journal = "The Canadian journal of cardiology",

issn = "0828-282X",

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TY - JOUR

T1 - Quality of Life in Adults with Transposition of the Great Arteries with a Systemic Right or Left Ventricle

AU - Ansari Ramandi, Mohammad Mostafa

AU - Van Bulck, Liesbet

AU - Ceelen, Daan C H

AU - Voors, Adriaan A

AU - Goossens, Eva

AU - Kovacs, Adrienne H

AU - Luyckx, Koen

AU - Van De Bruaene, Alexander

AU - Gabriel, Harald

AU - Lykkeberg, Birgitte

AU - Thomet, Corina

AU - de Hosson, Michèle

AU - Ladouceur, Magalie

AU - Saidi, Arwa

AU - Kosmidis, Diamantis

AU - Areias, Maria Emília

AU - Miranda, Joana

AU - Sandberg, Camilla

AU - Mandalenakis, Zacharias

AU - Coats, Louise

AU - Amedro, Pascal

AU - Khairy, Paul

AU - Valente, Anne Marie

AU - Johansson, Bengt

AU - Kaneva, Anna

AU - Andresen, Brith

AU - Christersson, Christina

AU - Reyes, Fernando Baraona

AU - Hlebowicz, Joanna

AU - Enomoto, Junko

AU - Kim, Yuli Y

AU - Menahem, Samuel

AU - Yang, Hsiao-Ling

AU - Moon, Ju Ryoung

AU - Bredy, Charlene

AU - Schmidt, André

AU - Callus, Edward

AU - Araujo, John Jairo

AU - Constantine, Andrew

AU - Zaidi, Ali

AU - Bouchardy, Judith

AU - Jameson, Susan M

AU - Kutty, Shelby

AU - McGrath, Lidija B

AU - Leong, Ming Chern

AU - Ortiz, Lucia

AU - Korkmaz, Fatma Demir

AU - Caruana, Maryanne

AU - Leye, Mohamed

AU - Moons, Philip

AU - APPROACH-IS II consortium

PY - 2025/12

Y1 - 2025/12

N2 - 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 TRIALS REGISTRATION: NCT04902768.

AB - 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 TRIALS REGISTRATION: NCT04902768.

KW - Adult

KW - Arterial Switch Operation/methods

KW - Congenitally Corrected Transposition of the Great Arteries/psychology

KW - Cross-Sectional Studies

KW - Female

KW - Heart Ventricles/physiopathology

KW - Humans

KW - Male

KW - Middle Aged

KW - Quality of Life

KW - Transposition of Great Vessels/psychology

KW - heart defects

KW - congenital

KW - mediation analysis

KW - systemic right ventricle

KW - quality of life

UR - http://www.scopus.com/inward/record.url?scp=105024871012&partnerID=8YFLogxK

U2 - 10.1016/j.cjca.2025.09.010

DO - 10.1016/j.cjca.2025.09.010

M3 - Journal article

C2 - 40962221

SN - 0828-282X

VL - 41

SP - 2544

EP - 2553

JO - The Canadian journal of cardiology

JF - The Canadian journal of cardiology

IS - 12

ER -

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

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