Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease
Retrospective cohort study in 60 patients on dual prostanoid therapy at maximal doses, found addition of a third agent improved 6-minute walking test distance (406 vs 450m, p=0.0027) at 1 year. Additionally, 79% had improvements in disease functional class at 1 yr & 76% at 2 yrs.
Source:
Heart
SPS commentary:
A related commentary discusses this research, stating that the safety profile of triple therapy in this study was also acceptable, with a low rate of side effects and a medication withdrawal. Also, there was a better response to PAH therapy observed in pretricuspid defects and in patients without Eisenmenger syndrome, emphasising the differences among groups. Moreover, a significant improvement was maintained just in patients treated with parenteral prostanoids, not in patients on oral selexipag or inhaled iloprost, underlying the different strength and efficacy of the different therapeutic approaches.
Commentary authors conclude that a number of open questions are still waiting for an answer for the management of these patients. For example, is oxygen administration useful in some patients, and is oral anticoagulation recommended?