Beyond Anatomical Severity: Determinants of Health-Related Quality of Life and Transition Readiness in Adolescents with Congenital Heart Disease

Background: Improved survival of adolescents with congenital heart disease has shifted the focus to examine health-related quality of life and address challenges in transition to adult care. We aim to describe how congenital heart disease complexity, gender, number of interventions, and Fontan circulation may affect the health-related quality of life and transition readiness of adolescents with congenital heart disease. Methods: We conducted a single-center cross-sectional study involving 536 patients aged 14 to 18 years old who attended a nurse-led, pediatric to adult care cardiac transition clinic, from 2020 to 2024. health-related quality of life was evaluated using the PedsQLTM 4.0 Generic Core Scales and the PedsQLTM 3.0 Cardiac Module. Patients were screened for anxiety and depression using the PHQ-9 and GAD-7. Transition readiness was assessed using the Transition-Q score. Results: The median age of patients was 16 years old and 44% self-identified as female. PedsQLTM 4.0 Generic had a median overall score of 77 (IQR 67?87), with no significant difference according to congenital heart disease severity. Female patients had significantly lower overall PedsQLTM 4.0 score (p=0.028) and lower physical and emotional functioning scores (p=0.005, p<0.001, respectively) when compared to males. Physical functioning scores were lower amongst patients with Fontan circulation compared to non-Fontan patients (p=0.003), although overall PedsQLTM 4.0 score and transition readiness scores were similar to those with complex biventricular congenital heart disease. Number of previous interventions were inversely associated with overall PedsQLTM 4.0 score (p=0.036). Moderate to severe symptoms of depression or anxiety were reported in 30% of screened patients and were associated with 2 significantly lower PedsQLTM 4.0 scores (p<0.001). Transition readiness was significantly lower in patients with moderate and complex compared to those with simple congenital heart disease (p<0.001). Transition readiness improved with repeat transition clinic visits (p=0.004) whereas PedsQLTM 4.0 score did not change significantly. Conclusion: In this large cohort of adolescents with congenital heart disease, health-related quality of life was lower than population norms. Female gender, higher interventional burden, and anxiety or depressive symptoms are associated with lower health-related quality of life scores rather than anatomical severity or Fontan physiology. Transition readiness was lower in complex disease; it has improved with a structured, nurse-led transition clinic, demonstrating modifiability. Consequently, adolescent congenital heart disease care requires a multidisciplinary approach including psychosocial screening, especially for high-risk groups, and structured transition planning to improve long-term outcomes.