Pediatric Pulmonary Arterial Hypertension; is it Possible to Predict its Outcome?

Objectives: To assess the outcome of pediatric pulmonary arterial hypertension (PAH) and to identify the predictors of morbidity and mortality of this progressive disease. Patients and methods: This prospective observational cohort study was conducted on consecutive pediatric patients with PAH. Medical history was taken with a grading of the WHO functional class as well as the serum N-terminal pro-BNP (NT pro-BNP), 6 min' walk test (6MWT), and echocardiography at the initial assessment and at follow-up. Results: The cohort study included 71 patients; 39 patients had idiopathic and heritable PAH, 27 patients had PH secondary to CHD and five patients had miscellaneous causes. Dyspnea was the most common presenting symptom. The mean initial 6MWT distance was 273.3 ± 139.9 m. The median of the initial NT-proBNP level was 1982 pg/ml with IQR from 373.9 to 5472 pg/ml. Death occurred in 17 patients (23.9%) and 16 (22.5%) had evolving morbidity. The predictors of morbidity were NT-proBNP >1032 pg/ml and its Z score > +3.5, WHO functional class 3 and 4, RV failure and low TAPSE. The predictors of mortality were 6MWD ≤210 m, WHO functional class 4, RV failure, syncope, low TAPSE, NT-proBNP >4734 pg/ml and its Z score >4.57. Conclusion: PAH is a serious disease and reliable predictive outcome is feasible by clinical assessment, echocardiographic parameters. NT pro-BNP is a surrogate biomarker for diagnosis and prognosis of PAH.