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Published on: February 3, 2014
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.
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.
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