Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease

Introduction and objectives: Outcome in patients with congenital heart diseases and pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Two-dimensional echocardio-graphic parameters, such as strain imaging or RV end-systolic remodeling index (RVESRI) have emerged to quantify RV function.Methods: We prospectively studied 30 patients aged 48 & PLUSMN; 12 years with pretricuspid shunt and PAH and investigated the accuracy of multiple echocardiographic parameters of RV function (tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity, RV systolic-to-diastolic duration ratio, right atrial area, RV fractional area change, RV global longitudinal strain and RVESRI) to RV ejection fraction measured by cardiac magnetic resonance.Results: RV ejection fraction < 45% was observed in 13 patients (43.3%). RV global longitudinal strain (p [Spearman's correlation coefficient] =-0.75; P = .001; R2 = 0.58; P = .001), right atrium area (p =-0.74; P < .0001; R2 = 0.56; P < .0001), RVESRI (p =-0.64; P < .0001; R2 = 0.47; P < .0001), systolic-to-diastolic duration ratio (p =-0.62; P = .0004; R2 = 0.47; P < .0001) and RV fractional area change (p = 0.48; P = .01; R2 = 0.37; P < .0001) were correlated with RV ejection fraction. RV global longitudinal strain, RVESRI and right atrium area predicted RV ejection fraction < 45% with the greatest area under curve (0.88; 95%CI, 0.71-1.00; 0.88; 95%CI, 0.76-1.00, and 0.89; 95%CI, 0.77-1.00, respectively). RV global longitudinal strain >-16%, RVESRI & GE; 1.7 and right atrial area & GE; 22 cm2 predicted RV ejection fraction < 45% with a sensitivity and specificity of 87.5% and 85.7%; 76.9% and 88.3%; 92.3% and 82.4%, respectively.Conclusions: RVESRI, right atrial area and RV global longitudinal strain are strong markers of RV dysfunction in patients with pretricuspid shunt and PAH. ⠅(C) 2022 Sociedad Espanola de Cardiologia. Published by Elsevier Espana, S.L.U. All rights reserved.