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

BackgroundAs 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. MethodsWe 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. ResultsPedsQL 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; Cramer's V = 0.10, 95% CI [0.06, 0.15]). At the 15th percentile, worse HRQL was associated with severe CHD ({approx} -5.9 points), poor transition support ({approx} -11.2), mood disorder ({approx} -12.7), and diabetes ({approx} -16.6). At the 85th percentile, severe CHD ({approx} -5.2) and mental-health comorbidity (mood {approx} -7.3; anxiety {approx} -5.6) was associated with worse HRQL. In severe CHD, poor transition support and mood or anxiety disorders (OR {approx} 0.48, 0.30, and 0.36, respectively; p [&le;] 0.01) markedly increased the odds of worse HRQL and reduced the odds of better HRQL. Very well-supported transition (OR {approx} 1.91, posterior probability = 0.99) was associated with better HRQL. ConclusionIn 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. What is Known; What the Study AddsO_ST_ABSWhat is KnownC_ST_ABSO_LIAs survival into adulthood increases for congenital heart disease (CHD) patients, health-related quality of life (HRQL) is an important outcome, yet adult-focused evidence remains limited. C_LIO_LIPrior studies rely on mean-based analyses that obscure patients doing particularly poorly or well and are confounded by healthcare-system differences. C_LI What the Study AddsO_LIThis is the first study in adult congenital heart disease (ACHD) research to use distribution-aware statistics (quantile regression and Bayesian proportional-odds models) to assess health-related quality of life (HRQL) beyond average scores and simple cutoffs to highlight risks and protective factors that may otherwise be missed. C_LIO_LIPoor transition support, mental-health comorbidity, diabetes, and regional or remote residence were key drivers of very low HRQL beyond anatomical severity, indicating ACHD-specific needs rather than disparities in healthcare access within Australias universal Medicare system. C_LIO_LIClinical take-home: prioritize structured transition, integrated mental-health care, diabetes management, and equitable access for regional/remote patients, using distribution-aware targeting to identify those at greatest risk of worse HRQL. C_LI