Risk of alveolar hemorrhage in patients with primary pulmonary hypertension: Anticoagulation and epoprostenol therapy

Background: Anticoagulation therapy and continuous intravenous infusion of epoprostenol are the standard treatment for primary pulmonary hypertension (PPH). Because epoprostenol has an antiplatelet effect, concomitant use of an anticoagulant could increase the likelihood of hemorrhagic complications. Methods and Results: In the present study, 31 consecutive patients with PPH (10 men, 21 women, mean±SD age, 28.5±10.1 years) treated with anticoagulation and epoprostenol between April 1999 and December 2003 were retrospectively evaluated. Clinical and hematological data at the time of the bleeding episode were retrieved from the medical records. Nine patients (22.6%) experienced 11 bleeding episodes: 9 episodes (81.8%) were alveolar hemorrhage and 2 patients were in severe respiratory distress. The mean dose of epoprostenol at the time of the first bleeding episode was 89.0±40.5 ng·kg^-1·min^-1 (range, 28.1-164.0). More of the patients who did not have a bleeding episode remain alive than did patients with bleeding episodes (59% vs 33%) nor did they require lung transplantation. Conclusions: A considerable number of patients with PPH who received combined anticoagulant and high-dose epoprostenol therapy developed alveolar hemorrhage, which can be fatal.