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Pediatric Pulmonary Arterial Hypertension; is it Possible to Predict its Outcome?
Hala M Agha1, Ranya Hegazy1, Ahmed Gamal1
1Department of Pediatrics, Pediatric Cardiology Division, Specialized Pediatric Hospital, Cairo University, Egypt.
Insights
Pediatric pulmonary arterial hypertension (PAH) is serious. Clinical assessment, echocardiography, and NT-proBNP levels predict outcomes in children with PAH, aiding diagnosis and prognosis.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Biomarkers
Background:
- Pediatric pulmonary arterial hypertension (PAH) is a progressive and severe condition.
- Accurate assessment of morbidity and mortality predictors is crucial for managing pediatric PAH.
Purpose of the Study:
- To evaluate the outcomes of pediatric pulmonary arterial hypertension (PAH).
- To identify key predictors of morbidity and mortality in pediatric PAH patients.
Main Methods:
- A prospective observational cohort study of 71 pediatric PAH patients.
- Data collection included medical history, WHO functional class, NT-proBNP levels, 6-minute walk test (6MWT), and echocardiography.
- Initial assessments and follow-up data were analyzed.
Main Results:
- Seventeen patients (23.9%) died, and 16 (22.5%) experienced evolving morbidity.
- Predictors of morbidity included elevated NT-proBNP, WHO functional class 3-4, RV failure, and low TAPSE.
- Predictors of mortality included reduced 6MWD, WHO functional class 4, RV failure, syncope, low TAPSE, and significantly elevated NT-proBNP levels.
Conclusions:
- Clinical assessment and echocardiographic parameters reliably predict outcomes in pediatric PAH.
- N-terminal pro-BNP (NT-proBNP) serves as a valuable surrogate biomarker for PAH diagnosis and prognosis.
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.
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