ArticlePulmonary circulation
Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review.
Article in Pulmonary circulation. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 145 papers, 5 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
145 citing papers in PubMed, 5 syntheses or guidelines pooled it.
- Quality of life in patients with pulmonary arterial hypertension: A systematic review and meta-analysis.Health and quality of life outcomes · 2026Pooled it
- Methodological Quality of Pulmonary Arterial Hypertension Treatment Evidence-Based Guidelines: A Systematic Review Using the AGREE II and AGREE REX Tools.Cardiovascular drugs and therapy · 2025Pooled it
- Screening for Pulmonary Hypertension in Connective Tissue Diseases: Literature Review and Multidisciplinary Consensus Statement.Anatolian journal of cardiology · 2025Pooled 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
- Pooled it
- Efficacy and safety of selexipag in patients with inoperable or persistent/recurrent CTEPH (SELECT randomised trial).The European respiratory journal · 2024Trial
- Safety, Tolerability, and Pharmacokinetics of Treprostinil Palmitil Inhalation Powder for Pulmonary Hypertension: A Phase 1, Randomized, Double-Blind, Single- and Multiple-Dose Study.Advances in therapy · 2022Trial
- 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
- Effect of supplemental oxygen therapy on 6-min walk distance in patients with pulmonary arterial hypertension living at 2850 m: a randomised, crossover trial.ERJ open research · 2026Article
- Polygonum cuspidatum Exosome-Like Nanovesicles Alleviate Hypoxic Pulmonary Hypertension by Stabilizing PON1 to Inhibit MAPK-Mediated PASMC Phenotypic Switching.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2026Article
- GLSP ameliorates monocrotaline-induced pulmonary arterial hypertension through inhibition of the PI3K/AKT and MAPK signaling pathways.Journal of molecular histology · 2026Article
- Ventricular arrhythmogenesis in pulmonary hypertension: From right ventricular remodeling to clinical implications.Physiological reports · 2026Review
- Article
- Socioeconomic Burden of Pulmonary Arterial Hypertension in Patients and Caregivers: A Canadian Cross-Sectional Study.CJC open · 2026Article
- Clinical Practice of Pulmonary Arterial Hypertension (PAH) and Potential Barriers to Intensive Treatment Among PAH-Specialized and Non-PAH-Specialized Centers in Japan.Pulmonary circulation · 2026Article
- Safety of exercise after pulmonary endarterectomy: a systematic review.ERJ open research · 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
- Evolution of Exercise Training in Patients with Pulmonary Hypertension-A Comprehensive Review.Healthcare (Basel, Switzerland) · 2026Review
- Observational
- Endothelial-to-Mesenchymal Transition Mechanisms in Vascular Remodeling of Pulmonary Hypertension.International journal of molecular sciences · 2026Review
85 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
This systematic review of literature and online reports critically appraised incidence and prevalence estimates of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension to identify the most accurate estimates. Medline® and Embase® databases were searched for articles published between 1 January 2003 and 31 August 2020. Studies were grouped according to whether they were registries (population-based estimates), clinical databases (hospital-based estimates) or claims/administrative databases. Registries were classified into systematic and non-systematic registries, according to whether every national centre participated. Of 7309 publications identified, 5414 were screened after removal of duplicates and 33 were included. Inclusion was based on study type, availability of a clear numerator (diagnosed population) and a population- or hospital-based denominator, or all primary data required to calculate estimates. Only the most recent publication from a database was included. Most studies were based on European data and very few included children. In adults, the range of estimates per million was approximately 20-fold for pulmonary arterial hypertension incidence (1.5-32) and prevalence (12.4-268) and of similar magnitude for chronic thromboembolic pulmonary hypertension incidence (0.9-39) and prevalence (14.5-144). Recent (≤5 years) national systematic registry data from centralised healthcare systems provided the following ranges in adult estimates per million: approximately 5.8 for pulmonary arterial hypertension incidence, 47.6-54.7 for pulmonary arterial hypertension prevalence, 3.1-6.0 for chronic thromboembolic pulmonary hypertension incidence and 25.8-38.4 for chronic thromboembolic pulmonary hypertension prevalence. These estimates were considered the most reliable and consistent for the scientific community to plan for resource allocation and improve detection rates.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.