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Clin Cardiol. 2018 Sep;41(9):1144-1149. doi: 10.1002/clc.22994. Epub 2018 Aug 18.

Right ventricular base/apex ratio in the assessment of pediatric pulmonary arterial hypertension: Results from the European Pediatric Pulmonary Vascular Disease Network.

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Division of Pediatric Cardiology, Department of Pediatrics, Medical University Graz, Graz, Austria.
Institute for Medical Informatics, Statistics and Documentation, Medical University Graz, Graz, Austria.
Department of Pediatric Cardiology, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Centre of Physiological Medicine, Medical University Graz, Graz, Austria.
Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany.



Echocardiographic determination of RV end-systolic base/apex (RVES b/a) ratio was proposed to be of clinical value for assessment of pulmonary arterial hypertension (PAH) in adults.


We hypothesized that the RVES b/a ratio will be affected in children with PAH and aimed to correlate RVES b/a ratio with conventionally used echocardiographic and hemodynamic variables, and with New York Heart Association (NYHA) functional class.


First we determined normal pediatric values for RVES b/a ratio in 157 healthy children (68 males; age range, 0.5-17.7 years). We then conducted an echocardiographic study in 51 children with PAH (29 males; age range, 0.3-17.8 years).


RVES b/a ratio was lower compared with age- and sex-matched healthy controls (P < 0.001). In children with PAH, RVES b/a ratio decreased with worsening NYHA class. RVES b/a ratio inversely correlated with RV/LV end-systolic diameter ratio (ρ = -0.450, P = 0.001) but did not correlate with RV systolic function parameters (eg, tricuspid annular plane systolic excursion) and correlated with cardiac catheterization-determined pulmonary vascular resistance index (ρ = -0.571, P < 0.001). ROC analysis unraveled excellent performance of RVES b/a ratio to detect PAH in children (AUC: 0.95, 95% CI: 0.89-1.00, P < 0.001).


The RVES b/a ratio decreased in children with PAH compared with age- and sex-matched healthy subjects. The RVES b/a ratio inversely correlated with both echocardiographic and hemodynamic indicators of increased RV pressure afterload and with NYHA class, suggesting that RVES b/a ratio reflects disease severity in PAH children.


Echocardiography; End-Systolic Base/Apex Ratio; Pediatric; Pulmonary Arterial Hypertension; Right Ventricle

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