Abstract 15610: Long Term Health Related Quality of Life Outcomes Across the Therapeutic Spectrum in Patients With Congenitally Corrected Transposition of Great Arteries

Abstract

Introduction: Congenitally corrected transposition of the great arteries (ccTGA) encompasses a diverse morphologic cohort with multiple treatment pathways. We characterized long term health related quality of life (HR-QoL) outcomes in adult patients with ccTGA across the therapeutic spectrum. Hypothesis: Methods: Between 1995-2020, 240 ccTGA patients were treated at the Cleveland Clinic. Of these, 59 adults were prospectively assessed with a hybrid PROMIS-10 and MacNew HR-QoL questionnaire at median follow-up of 10 years. Mental and physical scores were captured utilizing PROMIS 10 questionnaire, and social score from MacNew instrument. One-sample non-parametric sign tests evaluated deviations from referenced normative values. Differences among anatomic (AR) and physiologic repairs (PR) were assessed with Wilcoxon rank-sum test. Confidence intervals (95%) around measured means were generated from 1000 bootstrap samples. Results: Thirty one (18%) patients were female with median presentation age 26.3 years. Sixteen (27%) patients had PR, 12 (20%) had AR, 12 (20%) were medically managed, 5 (8.5%) had heart transplant and 3 (5.1%) underwent Fontan. Social scores were higher than the population norms (median= 6, P =.0002) for the overall cohort, but physical scores were lower than normative values (median = 44, P =.0183).Mental functioning was equivalent to normative values (median = 50, P =.9). AR and PR scores across domains were similar (Table 1). Conclusions: Adult patients living with ccTGA across the treatment spectrum have lower physical functioning than population norms. Despite these perceived physical impediments, social and mental functioning are unaffected. PR and AR had equivalent HR-QoL, providing important data to inform patient selection and triage.