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Published on: May 11, 2015
Prognostic factors in pediatric pulmonary arterial hypertension: A systematic review and meta-analysis
Mark-Jan Ploegstra1, Willemijn M H Zijlstra1, Johannes M Douwes1
1Centre for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, The Netherlands.
Insights
Prognosis in pediatric pulmonary arterial hypertension (PAH) remains poor. This study identified key prognostic factors, including WHO functional class and NT-proBNP, to improve treatment strategies and guidelines for children with PAH.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Clinical Epidemiology
Background:
- Pediatric pulmonary arterial hypertension (PAH) has a poor prognosis despite targeted therapies.
- There is a critical need for evidence-based prognostic factors to guide treatment strategies and clinical guidelines.
- Current understanding of prognostic indicators in pediatric PAH requires systematic evaluation.
Purpose of the Study:
- To systematically review and identify prognostic factors in pediatric PAH.
- To evaluate the prognostic value of reported factors using meta-analysis.
- To summarize evidence for clinical application in pediatric PAH management.
Main Methods:
- Systematic literature search of Medline, EMBASE, and Cochrane Library (up to April 2014).
- Inclusion of original studies reporting mortality or lung-transplantation predictors in pediatric PAH.
- Meta-analysis of Hazard Ratios (HR) and 95% Confidence Intervals for factors studied in ≥3 cohorts.
Main Results:
- 25 studies were included from 1053 identified citations.
- Significant prognostic factors identified: WHO functional class (HR 2.7), NT-proBNP (HR 3.2), mRAP (HR 1.1), PVRi (HR 1.3), and acute vasodilator response (HR 0.3).
- Cardiac index (HR 0.7) was also a significant prognostic factor.
Conclusions:
- WHO functional class, NT-proBNP, mRAP, PVRi, cardiac index, and acute vasodilator response are consistently reported prognostic factors in pediatric PAH.
- These validated factors can serve as valuable clinical tools for prognosis assessment.
- Incorporation of these prognostic factors into treatment strategies and guidelines is recommended for pediatric PAH.
Background:
Despite the introduction of targeted therapies in pediatric pulmonary arterial hypertension (PAH), prognosis remains poor. For the definition of treatment strategies and guidelines, there is a high need for an evidence-based recapitulation of prognostic factors. The aim of this study was to identify and evaluate prognostic factors in pediatric PAH by a systematic review of the literature and to summarize the prognostic value of currently reported prognostic factors using meta-analysis.
Methods And Results:
Medline, EMBASE and Cochrane Library were searched on April 1st 2014 to identify original studies that described predictors of mortality or lung-transplantation exclusively in children with PAH. 1053 citations were identified, of which 25 were included for further analysis. Hazard ratios (HR) and 95% confidence intervals were extracted from the papers. For variables studied in at least three non-overlapping cohorts, a combined HR was calculated using random-effects meta-analysis. WHO functional class (WHO-FC, HR 2.7), (N-terminal pro-) brain natriuretic peptide ([NT-pro]BNP, HR 3.2), mean right atrial pressure (mRAP, HR 1.1), cardiac index (HR 0.7), indexed pulmonary vascular resistance (PVRi, HR 1.3) and acute vasodilator response (HR 0.3) were identified as significant prognostic factors (p ≤ 0.001).
Conclusions:
This systematic review combined with separate meta-analyses shows that WHO-FC, (NT-pro)BNP, mRAP, PVRi, cardiac index and acute vasodilator response are consistently reported prognostic factors for outcome in pediatric PAH. These variables are useful clinical tools to assess prognosis and should be incorporated in treatment strategies and guidelines for children with PAH.
