Quality of Life in Adults With Transposition of the Great Arteries With a Systemic Right or Left Ventricle

Abstract

Background: Advances in medical and surgical care have improved survival in patients with transposition of the great arteries (TGA), shifting focus toward quality of life (QoL). In this study we evaluate QoL in adults with TGA, including congenitally corrected TGA (ccTGA) and patients with dextro-TGA (d-TGA), by comparing patients with a systemic right ventricle (sRV) and systemic left ventricle (sLV), while identifying mediating factors.

Methods: This cross-sectional study, part of the APPROACH-IS II trial, included 798 adults with TGA from 42 centres worldwide. QoL was assessed using a linear analogue scale (0-100). Regression models identified variables associated with QoL, and mediation analysis assessed the effect of sRV on QoL.

Results: Among participants (median age 34 years, 44.9% women), 504 (63.2%) had an sRV (ccTGA or d-TGA with atrial switch) and 294 (36.8%) had an sLV (ccTGA with double-switch operation or Rastelli and d-TGA with arterial switch or Rastelli). Patients with an sRV reported lower QoL (median 75, interquartile range 60-85) compared with those with an sLV (median 80, interquartile range 70-90; P < 0.001). The negative effect of sRV on QoL was mediated for 59% of patients by ventricular dysfunction (B = -2.37, 95% confidence interval -3.38 to -1.21; P < 0.001). Poorer QoL was independently associated with Asian race, employment status (job seeking, unemployed, or disabled), less social support, New York Heart Association functional class ≥ II, ventricular dysfunction, more interventional catheterizations, and depression/anxiety.

Conclusions: TGA patients with an sRV experience a lower QoL than those with an sLV, mediated mainly by ventricular dysfunction.

Clinical trial registration: NCT04902768.

Keywords: congenital; heart defects; mediation analysis; quality of life; systemic right ventricle.