Echocardiography in pediatric pulmonary arterial hypertension: early study on assessing disease severity and

Mark-Jan Ploegstra1, Marcus T R Roofthooft2, Johannes M Douwes1

  • 1From 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.

Insights

Echocardiography can assess disease severity and predict outcomes in pediatric pulmonary arterial hypertension (PAH). Key echocardiographic measures of both right and left heart function correlate with disease severity and survival in children with PAH.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Imaging
  • Pulmonary Hypertension Research

Background:

  • The prognostic value of echocardiography in pediatric pulmonary arterial hypertension (PAH) requires further definition.
  • Current understanding of echocardiography's role in assessing pediatric PAH severity and outcomes is limited.

Purpose of the Study:

  • To investigate the correlations between echocardiographic findings and disease severity in pediatric PAH.
  • To determine the relationship between echocardiographic parameters and patient outcomes in pediatric PAH.

Main Methods:

  • Prospective observational study of 43 children with idiopathic/hereditary PAH or PAH associated with congenital heart disease.
  • Echocardiographic assessment included 2D and Doppler measures of cardiac structure and function.
  • Correlations were analyzed between echocardiographic variables, disease severity markers (WHO-FC, NT-proBNP, hemodynamics), and lung-transplantation-free survival.

Main Results:

  • Right atrial and ventricular dimensions correlated with WHO-FC and hemodynamics.
  • Left ventricular dimensions were associated with hemodynamics and survival.
  • Right-to-left ventricular dimension ratio, right ventricular ejection time, and tricuspid annular plane systolic excursion all showed significant correlations with disease severity and/or survival.

Conclusions:

  • Echocardiographic characteristics of both right and left heart chambers are valuable indicators of disease severity and prognosis in pediatric PAH.
  • Echocardiography shows potential as a tool to guide management strategies for children diagnosed with PAH.
  • These findings support the use of echocardiography for monitoring disease progression and predicting outcomes in pediatric PAH patients.
Abstract

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