Abstract

BackgroundRight ventricular function has been identified as an important prognostic factor in children with pulmonary arterial hypertension. The aim of the study was to assess the deformation pattern and prognostic value of right ventricular longitudinal strain in children with pulmonary arterial hypertension.MethodsWe prospectively evaluated 46 children (25 with pulmonary arterial hyperetension and 21 age and sex matched controls) using conventional and speckle-tracking echocardiography, brain natriuretic peptide levels and clinical status expressed by WHO functional class and 6-min walking test. According to the clinical status after 12 months of follow-up, the pulmonary arterial hypertension patients were divided in two groups: non-worsening (13) and worsening (12).ResultsRight ventricular free wall longitudinal strain and strain rate were significantly lower in children with PAH compared with controls (−24.72 ± 3.48 vs −15.60 ± 3.40, p = 0.0001 and −1.44 ± 0.09 vs −1.09 ± 0.15, p = 0.0001, respectively). There was a more expressed decrease of basal than apical region of right ventricular free wall longitudinal strain/strain rate in pulmonary arterial hypertension patients compared with controls (strain: p = 0.0001 vs p = 0.07 and strain rate: p = 0.0001 vs p = 0.002). Comparing worsening and non-worsening pulmonary arterial hypertension patients there was a significant difference in Mid right ventricular free wall longitudinal strain (−14.00 ± 3.13 vs. −20.76 ± 4.62, p = 0.0001). In multivariable logistic regression analysis Mid right ventricular free wall longitudinal strain was an independent predictor of worsening in pulmonary arterial hypertension children (OR 0.45; 95 % CI: 0.21–0.96, p = 0.041). In ROC curve analysis a cut-off value of Mid right ventricular free wall longitudinal strain of −18.50 % predicted clinical worsening in pulmonary arterial hypertension children, with a sensitivity and specificity of 91.7 and 30.8 %, respectively (area under curve = 0.88 ± 0.06, 95 % CI: 0.75–1.00, p = 0.001).ConclusionsTwo-dimensional speckle-tracking echocardiography is a complementary non-invasive tool for assessment of right ventricular function in children with severe pulmonary arterial hypertension, allowing also clinical prediction and segmental analysis of right ventricular myocardial performance in these patients.

Highlights

  • Right ventricular function has been identified as an important prognostic factor in children with pulmonary arterial hypertension

  • The 6-min walk distance (6MWD), mean oxygen saturation (SpO2) before and after 6MWT were significantly lower in patients with Pulmonary arterial hypertension (PAH) compared with controls

  • More than half of the PAH children were in Word Health Organization (WHO) functional class 2

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Summary

Introduction

Right ventricular function has been identified as an important prognostic factor in children with pulmonary arterial hypertension. The aim of the study was to assess the deformation pattern and prognostic value of right ventricular longitudinal strain in children with pulmonary arterial hypertension. There are several clinical, laboratory and echocardiographic parameters that have been shown to be associated with higher risk of death in pediatric pulmonary hypertension, such as: Word Health Organization (WHO) functional class 3/4 [1, 4, 6], elevated levels of brain natriuretic peptide (BNP) [1, 7, 8] or evidence of right ventricular (RV) failure [1]. RV function has been identified as an important prognostic factor in patients with PAH regardless of the causal clinical entity [9, 10]. The recently developed speckle-tracking based strain imaging has been reported in adult patients as a technique that allows a better quantitative assessment of regional myocardial motion and deformation in PAH patients [18, 19], its usefulness in children has not been elucidated so far [14, 20, 21]

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