Systemic right ventricle (RV) is a rare and complex form of congenital heart disease (CHD) with a prognosis related to RV dysfunction and impaired physical capacity. Routine follow-up relies on echocardiography, however the prognostic value of echocardiography parameters remains under debate. Real-life patient follow-up involves different ultrasound systems. We aimed to evaluate echocardiography parameters’ reliability in systemic RV, in terms of reproducibility, using vendor-independent software, and in terms prediction of physical capacity impairment.

Adult patients with D-transposition of the great artery (d-TGA) who underwent atrial switch or with congenitally-corrected TGA (cc-TGA) were included in this multicentre prospective study. Current echocardiography parameters were analysed using TomTec-Arena™ software. Intraclass correlation coefficients (ICC) assessed inter- and intraobserver reliability. Associations between the most reproducible echocardiography parameters and exercise capacity (peak VO₂, VE/VCO₂ slope) were explored.

A total of 47 patients were included in the study (87% d-TGA, median age 36.4±8years). Conventional and 2D strain echocardiography parameters indicated the existence of a RV dysfunction (TAPSE=12.8±3.1mm; RV free wall longitudinal 2D strain=−13.6±3.9%). Good reproducibility (ICC>0.75) for both intra and interobserver variability was observed in 8 RV echocardiography parameters. Only the TAPSE was significantly associated with peak VO₂ (r=0.4, P=0.02).

In this prospective study mimicking real-life echocardiography follow-up of systemic RV, TAPSE, RV free wall longitudinal 2D strain and peak systolic S wave, were the most reproducible echocardiography parameters. However, only the TAPSE was associated with peak VO₂.