Key result
Only 9% of PAH patients receive guideline-recommended triple combination therapy at the time of death.
Why the study?
ESC/ERS Guidelines recommend parenteral prostacyclin analogues in combination therapy for high-risk PAH, but compliance with these guidelines at the last follow-up before death required verification.
Are PAH patients receiving guideline-recommended maximum medical therapy at the time of death?
Cohort (n=5,709)
Yes
Are PAH patients receiving guideline-recommended maximum medical therapy at the time of death?
Despite guideline recommendations, only a small minority of PAH patients in France and the UK receive maximum medical therapy (triple combination with parenteral prostacyclin) at the time of death.
Introduction ESC/ERS Guidelines recommend the use of parenteral prostacyclin analogues (PCA) in combination therapy in patients with pulmonary arterial hypertension (PAH) at high risk of death. The aim of the study was to describe the management of patients with PAH at the last follow-up before death to verify compliance with the ESC/ERS guidelines. Methods We examined the treatment received at the time of death in PAH patients from the French PH Registry and Imperial College London between 2009 and 2022. Results After a median follow-up of 2 years [IQR 0.4-4.7], 1924/4629 patients had died in the French cohort while 558/1080 patients died in the English cohort after 4.3 years [IQR 1.5-8.4]. Among the patients from the French PH registry who died, 9% (n = 160) were receiving triple combination therapy including IV/SC PCA at the time of death. This proportion was similar in the English cohort: 50/558 patients (9%). In both cohorts, 43% and 44% of patients received oral dual therapy, respectively. In the most recent period (2020-2022), 67% of patients received combination therapy, vs 48% before 2015, p < 0.001. In the French cohort, at the last follow-up visit (5 months before death), 7% were at low-risk, 23% at intermediate-low risk, 35% at intermediate-high risk and 35% at high-risk of death. Only 20% of patients at high-risk and 15% of patients at intermediate-high risk received triple combination therapy. Similar results were observed in the subset of patients with idiopathic / heritable or drug induced PAH. Conclusion Despite the different healthcare systems in France and the UK, only a minority of PAH patients were receiving the maximum medical treatment recommended in the guidelines at the time of their death.
No takes yet. Share an insight, caveat, or question.
Boucly et al. (2026) conducted a cohort in Pulmonary Arterial Hypertension (n=5,709). Triple combination therapy including IV/SC PCA vs. Other therapies was evaluated on Proportion of patients receiving triple combination therapy including IV/SC PCA at the time of death. At the time of death, only 9% of PAH patients in both the French and UK cohorts were receiving guideline-recommended triple combination therapy including parenteral prostacyclin analogues.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: