Initial triple therapy associated with improved long-term su
Initial triple combination therapy was associated with better survival among high-risk patients with severe pulmonary arterial hypertension at diagnosis, especially among younger high-risk patients, according to a new study.

The relationship between initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain.

This study aimed to evaluate long-term survival in PAH according to initial treatment strategy. Retrospective analysis of incident patients with idiopathic, heritable or anorexigen-induced PAH enrolled in the French Registry. Survival was assessed according to initial strategy: monotherapy, dual or triple combination (two oral medications and a parenteral prostacyclin).

-- Among 1611 enrolled patients, 984 were initiated with monotherapy, 551 with dual and 76 with triple therapy.

-- The triple combination group was younger with fewer comorbidities but higher mortality risk.

-- Survival was better with triple therapy (91% at 5 years) as compared to dual or monotherapy (both 61% at 5 years).

-- A propensity score matching on age, sex and pulmonary vascular resistance also showed significant differences between triple and dual therapy (10-year survival 85% vs 65%).

-- In high-risk patients (n=243), survival was better with triple therapy vs monotherapy or dual therapy, while there was no difference between monotherapy and double therapy.

-- In intermediate-risk patients (n=1134), survival improved with increasing number of therapies.

-- In multivariable Cox regression, triple therapy was independently associated with a lower risk of death.

-- Among the 148 patients initiated with a parenteral prostacyclin, those on triple therapy had better survival than those on monotherapy or dual therapy.

Conclusively, initial triple combination therapy including parenteral prostacyclin seems to be associated with better survival in PAH, particularly in the youngest high-risk patients.