Markers as predictors of clinical worsening in pulmonary arterial hypertension

Various biomarkers have been demonstrated to represent prognostic markers in adult patients with pulmonary arterial hypertension (PAH), however few data are available on their value in pediatric population. We aimed to assess the role of serum and imaging markers, such as B-type natriuretic peptide (BNP) and echocardiography-derived parameters characterizing right ventricular function and hemodynamics, in predicting clinical worsening in pediatric PAH. In 28 pediatric patients with PAH (group 1 - patients clinically stable at 12 months “no clinical worsening”, n = 14, 50%; group 2 - patients with clinical worsening at 12 months “clinical worsening”, n = 14, 50%), BNP, echocardiographic parameters (at baseline, 6 months and 12 months), World Health Organization functional class (“WHO FC”), 6-minute walk test (6MWT) were correlated with clinical status at 12 months. Baseline BNP levels were significantly higher in group 2 vs group 1 (47.19 pg/mL vs 16.97 pg/mL, p = 0.03) and presented an inverse non-significant correlation with 6MWT at baseline, 6 and 12 months. BNP significantly increased at 12 months in group 2 (118.03 pg/mL vs 12.97 pg/mL, p < 0.0001). ROC-analysis identified a baseline BNP cut-off value of 59.5 pg/mL with an 85.71% specificity (with an AUC of 0.71) for defining the group of patients at risk of clinical worsening from the group of patients who may have a stable clinical evolution. A BNP value > 59.5 pg/mL can serve as an indicator of high risk for future clinical deterioration in pediatric PAH.