Exercise Capacity and Mental Health in Adults With a Systemic Right Ventricle

Adults with a systemic right ventricle (sRV) due to congenitally corrected transposition of the great arteries (ccTGA) or atrial switch repair for d-transposition of the great arteries (d-TGA) experience substantial physiologic and psychosocial morbidity. Relationships among exercise capacity, sRV function, and mental health remain incompletely characterized.

Objectives

To characterize relationships among anatomic subtype, exercise capacity, sRV function, and mental health in adults with sRV physiology.

Methods

We performed a retrospective cohort study of adults with ccTGA or d-TGA (Mustard/Senning) followed at a tertiary Adult Congenital Heart Disease program from 2000 to 2025. Clinical, imaging, cardiopulmonary exercise testing, and patient-reported data were obtained from electronic health records. Mental health diagnoses were identified from clinical documentation. Functional status was assessed using NYHA class and the Kansas City Cardiomyopathy Questionnaire (KCCQ-12).

Results

Among 137 adults (ccTGA, n = 51; d-TGA, n = 86), percent-predicted peak VO2 was lower in d-TGA than ccTGA (60% vs 74%, p < 0.001), as was sRV systolic function (41 ± 11% vs 47 ± 10%, p < 0.01). Anxiety or depression was more common in d-TGA (46% vs 25%, p < 0.05). Across the cohort, anxiety or depression was associated with lower exercise capacity, worse NYHA functional class, and lower KCCQ scores.

Conclusions

Adults with d-TGA following atrial switch have lower exercise capacity, reduced sRV systolic function, and greater mental health burden than adults with ccTGA. These findings support integrated assessment of physiologic performance, functional status, and mental health in adults with sRV physiology.

Central Illustration. Integrated Cardio-Psychological Determinants of Exercise Capacity in Adults With a Systemic Right Ventricle. Adults with a sRV exhibit distinct clinical profiles according to anatomic subtype. Patients with d-TGA following atrial switch repair generally demonstrate lower exercise capacity, reduced systemic right ventricular systolic function, and a greater burden of anxiety, depression, and arrhythmias than patients with ccTGA. Exercise capacity reflects an integrated phenotype influenced by ventricular function, electrical disease, mental health, and functional health status. These findings support comprehensive assessment incorporating cardiac imaging, cardiopulmonary exercise testing, patient-reported health status, and mental health screening to guide multidisciplinary management and optimize functional performance and quality of life. Abbreviations: ccTGA = congenitally corrected transposition of the great arteries; CMR = cardiac magnetic resonance; CPET = cardiopulmonary exercise testing; d-TGA = d-transposition of the great arteries; KCCQ = Kansas City Cardiomyopathy Questionnaire; NYHA = New York Heart Association; sRV = systemic right ventricle; VO2 = peak oxygen consumption.