title: Echocardiography in pediatric pulmonary arterial hypertension: early study on assessing disease severity and predicting outcome.
abstract: 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.
  
  
  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.
authors.affiliation: 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.
authors.forename: Mark-Jan
authors.initials: MJ
authors.lastname: Ploegstra
authors.affiliation: 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. M.T.R.Roofthooft@UMCG.nl.
authors.forename: Marcus T R
authors.initials: MT
authors.lastname: Roofthooft
authors.affiliation: 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.
authors.forename: Johannes M
authors.initials: JM
authors.lastname: Douwes
authors.affiliation: 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.
authors.forename: Beatrijs
authors.initials: B
authors.lastname: Bartelds
authors.affiliation: 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.
authors.forename: Nynke J
authors.initials: NJ
authors.lastname: Elzenga
authors.affiliation: 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.
authors.forename: Dick
authors.initials: D
authors.lastname: van de Weerd
authors.affiliation: 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.
authors.forename: Hans L
authors.initials: HL
authors.lastname: Hillege
authors.affiliation: 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.
authors.forename: Rolf M F
authors.initials: RM
authors.lastname: Berger
mesh_terms: D000293:Adolescent; D000367:Age Factors; D016280:Atrial Function, Right; D062185:Atrial Pressure; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D015150:Echocardiography, Doppler; D065627:Familial Primary Pulmonary Hypertension / Q000000981:diagnostic imaging* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D007223:Infant; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D009426:Netherlands; D010446:Peptide Fragments / Q000097:blood; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013318:Stroke Volume; D013997:Time Factors; D014261:Tricuspid Valve / Q000000981:diagnostic imaging / Q000503:physiopathology; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right; D055815:Young Adult
chemical_list: D015415:Biomarkers; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: echocardiography; outcome measures; pediatrics; pulmonary hypertension