Right ventricular end-systolic remodeling index in the assessment of pediatric pulmonary arterial hypertension. The European Pediatric Pulmonary Vascular Disease Network (EPPVDN)

Background Echocardiographic determination of the right ventricular end-systolic remodeling index (RVES RI) has clinical value for the assessment of pulmonary hypertension (PH) in adults. We aim to determine RVES RI values in pediatric PH and to correlate RVES RI data with echocardiographic variables and NYHA functional class (FC). Methods Prospective echocardiography study in 49 children with PH. The 49 matched control subjects were chosen from 123 healthy children used to construct pediatric normal reference values. The associations with invasive hemodynamic variables were also investigated in a validation cohort of 12 PH children and matched controls. Results RVES RI was increased in children with PH vs. healthy controls (1.45 +/- 0.16 vs. 1.16 +/- 0.06; p < 0.01; confirmed in the validation cohort). RVES RI was associated with invasive hemodynamic variables, i.e. the mean pulmonary artery pressure. RVES RI values increased with worsening NYHA-FC. The highest RVES RI values were observed in PH children with NYHA FC 3 (1.60 +/- 0.12). Conclusions RVES RI is a useful indicator of RV remodeling and dilation in the setting of increased RV pressure load, especially when the degree of regurgitation of the tricuspid and pulmonary valves is insufficient to numerically estimate RV systolic pressure and mPAP, due to incomplete Doppler envelopes.