Abstract

Background Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. Methods Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. Results The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 +/- 6.4% vs. 48.5 +/- 7.2%, P = .81) and exercise capacity (% predicted peak VO2:82.5 +/- 17.7% vs. 75.6 +/- 20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF: r = -0.39, P = .04, rToF: r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF: r = -0.77, P < .001, rToF: r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 +/- 3.9 vs. -25.7 +/- 4.4, P < .001). Conclusions Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.