Pulmonary Arterial Hypertension (PAH) is a rare disorder that leads to increased pulmonary vascular resistance, with resulting dyspnea on exertion and fatigue, leading to right heart failure and death. Subcutaneous treprostinil has been shown to improve exercise tolerance over the short term, however long-term survival data are lacking.
The long term effects of treprostinil upon mortality were evaluated in 37 patients with severe PAH (mean pulmonary arterial pressure 57± 14 mmHg) taken from a single center. Most patients were women (92%), and the mean age was 47 ± 12 yrs. Survival to 5 years was assessed with Kaplan-Meier estimates, and compared to historical control survival predicted using the formula from the NIH Registry of primary pulmonary hypertension patients treated with conventional therapy.
The average follow-up time was 3.1 ± 2 years (range 12 days –5.0 years). The mean PAP, RAP, and CI were 57 ± 13 mmHg, 11.8 ± 5.1 mmHg, and 2.0 ± 0.6 L/min/m2 at baseline, respectively, reflecting severe pulmonary vascular disease. Kaplan-Meier curves showing survival probability for patients treated with treprostinil and for historical controls are shown in . Survival was improved compared to the expected survival predicted by the patients’ baseline hemodynamics. Figure 1
Long-term treprostinil is associated with improved survival for patients with severe PAH.
Subcutaneous treprostinil is an effective alternative to intravenous epoprostenol for the treatment of patients with severe PAH.
I. Okwuosa, None.