الفهرس 
Title : The Value Of N-terminal Pro-Brain Natriuretic Peptide In Pediatric Pulmonary Hypertension; A Single Center Study
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Abstract
Background: Pulmonary arterial hypertension (PAH) is a devastating, progressive disease with increasingly debilitating symptoms and usually shortened overall life expectancy due to narrowing of the pulmonary vasculature and consecutive right heart failure (RHF).
Aim of the work: to assess the severity, the prognosis, and the outcome of PAH and to identify the predictors of morbidity and mortality of this serious disease in the pediatric population.
Patients and methods: This is a prospective observational cohort study conducted on all pediatric patients with pulmonary arterial hypertension presented to Pediatric Cardiology Division, Cairo University from January 2020 to September 2022. All patients were subjected to full medical history, general and cardiac examination, assessment of the WHO functional class, N-terminal pro-BNP, 6MWT and echocardiography during the initial assessment and the follow up.
Results:Cohort study included 106 patients; 30 of them (28.3%) were idiopathic PAH, 27 patients (25.5%) were secondary to CHD, 35 patients (33%) were due to restrictive lung disease, and 13.2% due to other causes. Dyspnea was the most common presenting symptom. The mean value of 6MWD was 299.3 ± 148.7 meters. The NT pro-BNP level was significantly high; median level =851.5 pg/ml [IQR=220- 5140.1 pg/ml]. In our series, there was a significant positive correlation between the NT pro-BNP and z score of the right ventricular end-diastolic diameter and z score of the right atrial area. We reported 22 mortality cases (19.6%) and 15 morbidity cases (13.4%). The predictors of morbidity were; WHO functional classes (III and IV), the recurrent syncopal attacks, the presence of RV failure, and the right ventricular dilatation (RV/LV ratio ≥ 0.93), the right atrial dilatation (z score of RA area ≥ 2.64) and RVOT acceleration time (≤74 milliseconds) and the low 6MWD (a distance ≤190 meters). High NT pro-BNP level; a cut-off value of 1032 pg/ml was considered a predictor of morbidity and a level of 4830pg/ml was a predictor of mortality in our series.
Conclusion: Pediatric pulmonary hypertension is a serious disease with high mortality and morbidity. Prediction of the outcome is feasible by clinical assessment, echocardiographic parameters and NT pro-BNP as biomarker for diagnosis and prognosis.