Rationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension PAH remains uncertain. Objectives: To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. Methods: A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry January to December was conducted. Survival was assessed according to the initial strategy: monotherapy, dual therapy, or triple-combination therapy two oral medications and a parenteral prostacyclin.
Measurements and Main Results: Among 1, enrolled patients, were initiated on monotherapy, were initiated on dual therapy, and 76 were initiated on triple therapy. The triple-combination group was younger and had fewer comorbidities but had a higher mortality risk. In multivariable Cox regression, triple therapy was independently associated with a lower risk of death hazard ratio, 0.
Among the patients initiated on a parenteral prostacyclin, those on triple therapy had a higher survival rate than those on monotherapy or dual therapy. Conclusions: Initial triple-combination therapy that includes parenteral prostacyclin seems to be associated with a higher survival rate in PAH, particularly in the youngest high-risk patients. Keywords: pulmonary arterial hypertension; pulmonary hypertension; survival; therapeutics.
Abstract Rationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension PAH remains uncertain.
Publication types Evaluation Study. Substances Antihypertensive Agents.
