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Advisor(s)
Abstract(s)
Background: Higher quality scaled-up evidence on pulmonary arterial hypertension (PAH), a rare and life-threatening disease, is needed to support informed decision-making. We aimed to map the current knowledge of PAH treatments and evaluate the methodological quality of published systematic reviews. Methods: An overview with literature searches in PubMed and Embase (May 2025) was performed (CRD42023414469). The methodological and reporting quality of the eligible records was assessed using the Measurement Tool to Assess Systematic Reviews (AMSTAR 2) and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 checklist, respectively. Association analyses between tools' scores with key variables (article publication date, journal impact factor, country/region) were performed (SPSS v.28). An evidence map summarizing the most reported treatments and outcomes was also built. Results: Overall, 57 systematic reviews (n = 52; 91.2% with meta-analysis) published between 1997 and 2025 (median year 2017), authored mostly by countries from Asia (n = 35; 61.4%) and North America (n = 12; 21.1%), were included. The classes of phosphodiesterase type-5 inhibitors, endothelin receptor antagonists, prostanoids, and combination therapies were each assessed in one-third of the studies. Over 20 different outcomes were reported, with the most common surrogate endpoints being 6-minute walking distance (n = 42; 73.7%) and mean pulmonary arterial pressure (n = 33; 57.9%). Most studies were classified as having critically low methodological quality (n = 48; 84.2%), with only three presenting high-quality methodology according to AMSTAR 2. The mean PRISMA score was 21.3 ± 2.9, indicating an adherence rate of 78.9% to the checklist among authors. Although there was an improvement over time in the quality of the reviews (p = 0.016 for AMSTAR; p = 0.002 for PRISMA), no correlations were found based on country or journal impact factor. Conclusions: Methodological weaknesses remain common in systematic reviews of PAH; therefore, enforcing compliance with guidelines and standardizing outcome measurements through a core outcome set is crucial for improving data comparability and clinical application. Registration: PROSPERO identifier no. CRD42023414469.
Description
This study was supported by the Coordenação de Aperfeiçoamento de Pessoal de Nível Superior—Brasil (CAPES)—Finance Code 001. The funding sources had no role in the design and conduct of the study, the collection, management, analysis, and interpretation of the data, the preparation, review, or approval of the manuscript, or the decision to submit the manuscript for publication.
Keywords
Pulmonary arterial hypertension Systematic review Methodological quality Evidence gaps
Pedagogical Context
Citation
Vilela AP, Deffert F, Tonin FS, Pontarolo R. Overview of systematic reviews on treatments for pulmonary arterial hypertension: assessing methodological quality and mapping evidence gaps. Am J Cardiovasc Drugs. 2025;25(6):779-802.
Publisher
Springer Science and Business Media LLC
