Health-Related Quality of Life in Adults With Congenital Heart Disease: A Population-Based Study.

BACKGROUND: As survival into adulthood improves for patients with congenital heart disease (CHD), quality of that survival is paramount. We assessed health-related quality of life (HRQL) in a large adult CHD cohort. METHODS: We studied 868 participants from a multicenter registry with complete Pediatric Quality of Life Inventory 4.0 Generic Core Scale and covariate data. Analyses followed 3 stages: CHD complexity across HRQL score bands, predictors of worse or better HRQL, and predictors of HRQL in severe CHD. RESULTS: Pediatric Quality of Life Inventory total scores were high (median 76.1, interquartile range 64.1-87.0), highest in Social functioning, with longer lower tails for Emotional and Psychosocial scales. Severe CHD was over-represented in the lower and under-represented in the upper 15% bands (Total HRQL: P<0.001). At the 15th percentile, worse HRQL was linked to severe CHD (-5.9; 95% CI -11.6 to -0.1), poor transition support (-11.2; 95% CI -18.6 to -3.9), mood disorder (-12.7; 95% CI -18.3 to -7.1), and diabetes (-16.6; 95% CI -29.9 to -3.3); at the 85th percentile, to severe CHD (-5.2; 95% CI -9.2 to -1.1), mood disorder (-7.3; 95% CI -11.7 to -3.0), anxiety disorder (-5.6; 95% CI -9.7 to -1.5). In severe CHD, poor transition support and mood or anxiety disorders (odds ratio [OR] 0.48, 0.30, and 0.36, respectively; P=0.01) markedly increased the odds of worse HRQL. Very well-supported transition (OR 1.91) was associated with better HRQL. CONCLUSIONS: In adult CHD, anatomic complexity contributes modestly to HRQL extremes. Modifiable predictors (transition experience, mental-health comorbidity, location, and diabetes) emerge as key targets to improve HRQL.