• Mark-Jan Ploegstra, Marcus T R Roofthooft, Johannes M Douwes, Beatrijs Bartelds, Nynke J Elzenga, Dick van de Weerd, Hans L Hillege, and Rolf M F Berger.
• From the Center for Congenital Heart Diseases, Dutch National Referral Center for Children with Pulmonary Hypertension, Beatrix Children's Hospital (M.-J.P., M.T.R.R., J.M.D., B.B., N.J.E., D.v.d.W., R.M.F.B) and Department of Epidemiology (H.L.H), University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
• Circ Cardiovasc Imaging. 2015 Jan 1;8(1).

BackgroundThe 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 ResultsForty-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).ConclusionsThis 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.© 2014 American Heart Association, Inc.

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