Long Term Health Related Quality Of Life Outcomes Across The Therapeutic Spectrum In Patients With Congenitally Corrected Transposition Of Great Arteries: A Balancing Score
Sohini Gupta, Dominique L. Tucker, Olivia McCloskey, Chang Yoon Doh, Miza Salim Hammoud, MD, Andrew Toth, MS, Paola A. Barrios, Rukmini Komarlu, Gosta Pettersson, Eugene Blackstone, Hani K.Najm, Tara Karamlou.
Cleveland Clinic, Cleveland, OH, USA.
Objective(s): We characterized long term health related quality of life (HR-QoL) outcomes in adult patients with congenitally corrected transposition of the great arteries (ccTGA) across the therapeutic spectrum.
Methods: Between 1995-2020, 240 ccTGA patients were treated at the Cleveland Clinic. Of these, 59 adults were prospectively assessed with hybrid PROMIS-10 and MacNew HR-QoL questionnaire at 10 year median follow-up. Mental and physical scores were captured utilizing PROMIS 10 questionnaire, and social score from MacNew. One-sample non-parametric sign tests evaluated deviations from referenced normative values. Wilcoxon rank-sum test assessed differences among anatomic (AR) and physiologic repairs (PR).Results: Median presentation age was 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 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). Physical scores tended to be higher among AR compared to PR (49 compared to 43.9 respectively, P= .066,) but the remaining scores were similar(Figure 1).
Conclusions: Long-term HR-QoL data can inform ccTGA patient selection and triage across the therapeutic spectrum. Minimal HR-QoL gains among adult survivors with anatomical repair of ccTGA may dampen enthusiasm for proactive management of ‘balanced' ccTGA towards AR. 
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