Echocardiography in Pediatric Pulmonary Arterial Hypertension

Background— The value of echocardiography in assessing disease severity and predicting outcome in pediatric pulmonary arterial hypertension (PAH) is insufficiently defined. The aim of this study was to describe correlations between echocardiography and disease severity and outcome in pediatric PAH. Methods and Results— Forty-three consecutive children (median age, 8.0 years; range, 0.4–21.5) with idiopathic/hereditary PAH (n=25) or PAH associated with congenital heart disease (n=18) were enrolled in a prospective single-center observational study. Anatomic and right ventricular-functional variables were obtained by two-dimensional echocardiography and Doppler-echocardiography at presentation and at standardized follow-up and were correlated with measures of disease severity (World Health Organization functional class [WHO-FC], N-terminal-pro-B-type natriuretic peptide, hemodynamics) and lung-transplantation–free survival. Right atrial and right ventricular dimensions correlated with WHO-FC and hemodynamics ( P <0.05), whereas left ventricular dimensions correlated with hemodynamics and survival ( P <0.05). Right-to-left ventricular dimension ratiocorrelated with WHO-FC, hemodynamics and survival ( P <0.05). Right ventricular ejection time correlated with hemodynamics and survival ( P <0.05) and tended to correlate with WHO-FC ( P =0.071). Tricuspid annular plane systolic excursion correlated with WHO-FC, mean right atrial pressure and survival ( P <0.05). Conclusions— This early descriptive study shows that echocardiographic chararacteristics of both the right and the left heart correlate with disease severity and outcome in pediatric PAH, both at presentation and during the course of the disease. The preliminary data from this study support the potential value of echocardiography as a tool in guiding management in children with PAH.