Health-Reported Quality of Life in Patients with A Systemic Right Ventricle: The QUALISYSTEMIC Study

Structured Abstract Background The systemic right ventricle (SRV) represents one of the most complex congenital heart diseases (CHD), involving two main anatomical presentations: D-looped transposition of the great arteries and congenitally corrected transposition of the great arteries. Recent therapeutic advances have attempted to improve prognosis in adults with an SRV, but the impact on health-related quality of life (HRQoL) has not been clearly established. Methods Adult patients aged 18 years with an SRV were prospectively enrolled in a multicentre cross-sectional study with recruitment across 13 CHD centres. Demographic, clinical, biological, and imaging data were analysed to assess HRQoL and its determinants. Results A total of 99 patients (mean age, 38.7±9.2 years; 52% male) were included. Most were symptomatic (NYHA II: 52.2%; NYHA III: 33.7%) with reduced exercise capacity (mean VO₂max=20±6.1 ml/Kg/min, i.e., 62.4%±16.8% of predicted values). HRQoL showed moderate impairment with a physical component score of 45.9±8.7 and a mental component score of 46.2±9.5. A lower physical component score was independently associated with a reduced VO₂max, a recent episode of decompensated heart failure, vitamin K antagonist medication, and being unemployed. The use of antiarrhythmic medications, the number of heart-failure related treatments, and being unemployed were independently associated to a lower mental component score, but having undergone interventional cardiac catheterization was associated with a better mental component score. Conclusions HRQoL in adults with SRV shows moderate impairment compared to normative data. Cardiopulmonary fitness and clinical factors significantly influence physical and mental components of HRQoL, underscoring the importance of targeted interventions to optimize patient outcomes. ClinicalTrials.gov ID NCT03379831