ArticleThe European respiratory journal2022
Mortality trends in pulmonary arterial hypertension in Canada: a temporal analysis of survival per ESC/ERS guideline era.
Article in The European respiratory journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers, 1 of them a synthesis that pooled it.
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31 citing papers in PubMed, 1 synthesis or guideline pooled it, 61 citations in OpenAlex.
- 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
- GLSP ameliorates monocrotaline-induced pulmonary arterial hypertension through inhibition of the PI3K/AKT and MAPK signaling pathways.Journal of molecular histology · 2026Article
- Socioeconomic Burden of Pulmonary Arterial Hypertension in Patients and Caregivers: A Canadian Cross-Sectional Study.CJC open · 2026Article
- Associations of Red Blood Cell Distribution Width-Derived Indicators and Their Longitudinal Dynamic Trajectories With Mortality Risk in Critically Ill Patients With Pulmonary Hypertension.Pulmonary circulation · 2026Article
- Treatment of Pulmonary Arterial Hypertension in Lithuania: Current Situation and Analysis of Survival of Patients Treated with Different Treatment Regimens.Journal of clinical medicine · 2026Article
- Macitentan and phosphodiesterase-5 inhibitor alone or in combination in newly diagnosed pulmonary arterial hypertension: a pooled analysis.JHLT open · 2026Article
- Performance on the one-minute sit-to-stand test predicts long-term adverse outcomes in pulmonary hypertension.Scientific reports · 2026Article
- Upfront triple combination therapy with selexipag: insights from a real world cohort in Chinese patients with pulmonary arterial hypertension.Frontiers in cardiovascular medicine · 2026Article
- Advances in Novel Therapeutic Strategies for Pulmonary Arterial Hypertension.Canadian respiratory journal · 2026Review
- Sex differences in pulmonary (arterial) hypertension: does it matter?Current opinion in pulmonary medicine · 2025Review
- Sex differences in pulmonary (arterial) hypertension: does it matter?Current opinion in pulmonary medicine · 2025Review
- Exploratory Study of Prognostic Plasma Biomarkers in Patients with Pulmonary Arterial Hypertension.The American journal of pathology · 2025Article
- C-C Motif chemokine receptor-2 blockade ameliorates pulmonary hypertension in rats and synergizes with a pulmonary vasodilator.Cardiovascular research · 2025Article
- Lipid Ratios for Diagnosis and Prognosis of Pulmonary Hypertension.American journal of respiratory and critical care medicine · 2025Article
- A new day has come: Sotatercept for the treatment of pulmonary arterial hypertension.The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2025Review
- Implication of m6A Methylation Regulators in the Immune Microenvironment of Bronchopulmonary Dysplasia.Biochemical genetics · 2024Article
- Carotid Baroreceptor Stimulation Ameliorates Pulmonary Arterial Remodeling in Rats With Hypoxia-Induced Pulmonary Hypertension.Journal of the American Heart Association · 2024Article
- Update on Eisenmenger syndrome - Review of pathophysiology and recent progress in risk assessment and management.International journal of cardiology. Congenital heart disease · 2024Review
- Future Perspectives of Pulmonary Arterial Hypertension: A Review of Novel Pipeline Treatments and Indications.Drugs in R&D · 2024Review
- Elderly Patients with Idiopathic Pulmonary Hypertension: Clinical Characteristics, Survival, and Risk Stratification in a Single-Center Prospective Registry.Life (Basel, Switzerland) · 2024Article
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Authors and funding
19 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe evolution in pulmonary arterial hypertension (PAH) management has been summarised in three iterations of the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. No study has assessed whether changes in management, as reflected in the changing guidelines, has translated to improved long-term survival in PAH.
methodsWe performed a mixed retrospective/prospective analysis of treatment-naïve, incident PAH patients (n=392) diagnosed at three major centres in Canada from 2009 to 2021. Patients were divided into two groups based on their diagnosis date and in accordance with the 2009 and 2015 ESC/ERS guideline iterations. Overall survival was assessed based on date of diagnosis and initial treatment strategy (
resultsThere was a shift towards more aggressive upfront management with combination therapy in Canada after the publication of the 2015 ESC/ERS guidelines (10.4% and 30.8% in patients from 2009 to 2015 and 36.0% and 57.4% in patients diagnosed after 2015 for baseline and 2-year follow-up, respectively). A key factor associated with combination therapy after 2015 was higher pulmonary vascular resistance (p=0.009). The 1-, 3- and 5-year survival rates in Canada were 89.2%, 75.6% and 56.0%, respectively. Despite changes in management, there was no improvement in long-term survival before and after publication of the 2015 ESC/ERS guidelines (p=0.53).
conclusionsThere was an increase in the use of initial and sequential combination therapy in Canada after publication of the 2015 ESC/ERS guidelines, which was not associated with improved long-term survival. These data highlight the continued difficulties of managing this aggressive pulmonary disease in an era without a cure.
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