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

Abstract

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 echocardiographic 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±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 (ρ [Spearman's correlation coefficient]=-0.75; P=.001; R²=0.58; P=.001), right atrium area (ρ=-0.74; P <.0001; R²=0.56; P <.0001), RVESRI (ρ=-0.64; P <.0001; R²=0.47; P <.0001), systolic-to-diastolic duration ratio (ρ=-0.62; P=.0004; R²=0.47; P <.0001) and RV fractional area change (ρ=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 ≥ 1.7 and right atrial area ≥ 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.

Keywords: Cardiopatías congénitas; Congenital heart disease; Echocardiography; Ecocardiografía; Hipertensión pulmonar arterial; Pulmonary arterial hypertension; Right ventricle; Right ventricle end-systolic remodeling index; Ventrículo derecho; Índice de remodelado ventricular derecho telesistólico.