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

Advances in medical and surgical care have improved survival in patients with transposition of the great arteries (TGA), shifting focus towards quality of life (QoL). This study evaluates 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. This cross-sectional study, part of the APPROACH-IS II study, included 798 adults with TGA from 42 centers worldwide. QoL was assessed using a linear analogue scale (0-100). Regression models identified variables associated with QoL, and mediation analysis examined the effect of sRV on QoL. Among participants (median age 34 years, 44.9% women), 504 patients (63.2%) had an sRV (ccTGA or d-TGA with atrial switch), while 294 patients (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 (75 [IQR: 60-85]) compared to those with an sLV (80 [IQR: 70-90], p <0.001). The negative effect of sRV on QoL was mediated for 59% by ventricular dysfunction (B=-2.37, 95%CI: [-3.38- -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, a greater number of interventional catheterizations and depression/anxiety. TGA patients with an sRV experience lower QoL than those with an sLV, mediated mainly by ventricular dysfunction.