Health-Related Quality of Life in Adults with Congenital Heart Disease - A Population-Based Study from the Australian National Registry

Background: As survival into adulthood improves for congenital heart disease (CHD) patients, the quality of survival is paramount. We assessed health-related quality of life (HRQL) in a large Australian adult CHD cohort and identified clinical, psychosocial and system-level predictors of better or worse HRQL. Methods: We conducted a multicenter, registry-based cross-sectional study of a random stratified sample of adults from the Registry, assessing 868 participants who completed the PedsQL 4.0 Generic Core Scales and had complete covariate data. Analyses were structured into three stages: Stage I examined CHD complexity (mild, moderate, severe) across the lower (bottom 15%), middle (70%), and upper (top 15%) HRQL score bands; Stage II identified predictors of worse or better HRQL at the tails of the distribution; and Stage III assessed predictors of overall HRQL within the severe CHD group. Results: PedsQL Total scores were generally high (median 76.1, IQR 64.1-87.0) and best in Social functioning; Emotional and Psychosocial scales showed longer lower tails. Severe CHD was over-represented in the lower 15% and under-represented in the upper 15% bands (Total HRQL: p<0.001;[(Cramér's V= 0.10,95% CI [0.06,0.15]). At the 15th percentile, worse HRQL was associated with severe CHD (≈-5.9 points), poor transition support (≈-11.2), mood disorder (≈-12.7), and diabetes (≈-16.6). At the 85th percentile, severe CHD (≈-5.2) and mental-health comorbidity (mood ≈ -7.3; anxiety ≈-5.6) was associated with worse HRQL. In severe CHD, poor transition support and mood or anxiety disorders (OR≈0.48,0.30,and 0.36, respectively; p ≤ 0.01) markedly increased the odds of worse HRQL and reduced the odds of better HRQL. Very well-supported transition (OR≈1.91, posterior probability = 0.99) was associated with better HRQL. Conclusion: In adult CHD, anatomic complexity contributes modestly to HRQL extremes. Modifiable predictors (transition experience and mental-health comorbidity, location and diabetes) emerge as key targets to improve HRQL, particularly in severe CHD.