ArticlePulmonary circulation2022
Prevalence, incidence, and survival of pulmonary arterial hypertension: A systematic review for the global burden of disease 2020 study.
Article in Pulmonary circulation, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers, 3 of them syntheses that pooled it.
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Who cites it
45 citing papers in PubMed, 3 syntheses or guidelines pooled it, 71 citations in OpenAlex.
- Transitioning Prostacyclin Pathway Agents to Oral Selexipag in Patients with Pulmonary Arterial Hypertension: A Systematic Literature Review.Advances in therapy · 2026Pooled it
- Survival, morbidity, and quality of life in pulmonary arterial hypertension patients: a systematic review of outcomes reported by population-based observational studies.Respiratory research · 2024Pooled it
- Pulmonary hypertension survival and hospitalisations in people living with HIV: a systematic review and meta-analysis.BMJ open respiratory research · 2024Pooled it
- Effect of Famotidine on Outcomes in Pulmonary Arterial Hypertension: A Randomized Controlled Trial.Chest · 2025Trial
- Burden dynamics of pulmonary arterial hypertension in China and the G20 aggregate: a comparative analysis based on the global burden of disease study 2023.Annals of medicine · 2026Article
- Comparative clinical evaluation of endothelin receptor antagonists in pulmonary arterial hypertension using a multi-criteria decision analysis framework.International journal of clinical pharmacy · 2026Article
- The Clinical and Economic Burden of Pulmonary Arterial Hypertension in Israel, 2014-2022: A Retrospective Analysis of Healthcare Costs and Utilization.Pulmonary circulation · 2026Article
- Expert Consensus to Explore the Definition and Characterization of Methamphetamine-Associated Pulmonary Arterial Hypertension and Key Treatment Considerations.CHEST pulmonary · 2026Article
- Article
- Healthcare Resource Utilization and Medical Costs in Pulmonary Arterial Hypertension Management in Japan: A Retrospective Study Using a Claims Database.Pulmonary therapy · 2026Article
- Selection of Endothelin Receptor Antagonists in the Treatment of Pulmonary Arterial Hypertension: A Comprehensive Narrative Review.Advances in therapy · 2026Review
- The trend analysis and comparison of pulmonary arterial hypertension disease burden in China and India from 1990 to 2021: a study based on the Global Burden of Disease 2021 database.European journal of medical research · 2025Article
- Pulmonary arterial hypertension and the limits of convenience: a critical look at yutrepia.Annals of medicine and surgery (2012) · 2025Article
- Managing breathlessness for people with advanced respiratory disease.ERJ open research · 2025Review
- Exploring the uncharted role of cell senescence in rare diseases.Orphanet journal of rare diseases · 2025Review
- Global pulmonary arterial hypertension trends and projections to 2046: a multi-method analysis of epidemiologic and demographic drivers using GBD 2021.Journal of thoracic disease · 2025Article
- Self-supervised learning for label-free segmentation in cardiac ultrasound.Nature communications · 2025Article
- Genetic screening in cohort of Egyptian patients with pulmonary arterial hypertension disease.Irish journal of medical science · 2025Article
- Melatonin Improves Quality of Life, Oxidative Stress, and Cardiovascular Function in Pulmonary Arterial Hypertension.Pulmonary circulation · 2025Article
- Global distribution of prognostically significant pulmonary pressure indicative of pulmonary hypertension.Journal of global health · 2025Article
Corrections and comments
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Authors and funding
8 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary arterial hypertension (PAH) is characterized by increased resistance in the pulmonary arterioles as a result of remodeled blood vessels. We sought all available epidemiologic data on population-based prevalence, incidence, and 1-year survival of PAH as part of the Global Burden of Disease Study. We performed a systematic review searching Global Index Medicus (GIM) for keywords related to PAH between 1980 and 2021 and identified population-representative sources of prevalence, incidence, and mortality for clinically diagnosed PAH. Of 6772 articles identified we found 65 with population-level data: 17 for prevalence, 17 for incidence, and 58 reporting case fatality. Reported prevalence ranged from 0.37 cases/100,000 persons in a referral center of French children to 15 cases/100,000 persons in an Australian study. Reported incidence ranged from 0.008 cases/100,000 person-years in Finland, to 1.4 cases/100,000 person-years in a retrospective chart review at a clinic in Utah, United States. Reported 1-year survival ranged from 67% to 99%. All studies with sex-specific estimates of prevalence or incidence reported higher levels in females than males. Studies varied in their size, study design, diagnostic criteria, and sampling procedures. Reported PAH prevalence, incidence, and mortality varied by location and study. Prevalence ranged from 0.4 to 1.4 per 100,000 persons. Harmonization of methods for PAH registries would improve efforts at disease surveillance. Results of this search contribute to ongoing efforts to quantify the global burden of PAH.
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