Initial therapy in patients with pulmonary arterial hypertension and cardiovascular comorbidities.

Abstract

Background: European guidelines recommend initial monotherapy in PAH patients with cardiovascular (CV) comorbidities based on the limited of evidence for combination therapy in this growing population.

Methods: A retrospective analysis was conducted on incident PAH patients enrolled in the French Pulmonary Hypertension Registry between 2009 and 2020. Propensity score matching was used to investigate initial dual oral combination therapy versus oral monotherapy in patients with at least one CV comorbidity (i.e., hypertension, obesity, diabetes and coronary artery disease).

Results: Of the 1784 patients identified, 1088 had ≥1 CV comorbidity, including 20% with ≥3 comorbidities. In the propensity score matched population (N=708), the majority of patients were female, with idiopathic/heritable/drug-induced PAH, at intermediate 1-year mortality risk. At first follow-up, initial dual therapy led to larger improvements in symptoms, exercise capacity, hemodynamics, and risk status than initial monotherapy, with no differences in long-term survival. Treatment discontinuations were observed in 23% of patients initiated on dual therapy and 24% of those initiated on monotherapy.

Conclusions: Initial dual oral combination therapy may be beneficial and well tolerated in most PAH patients with CV comorbidities.