PURPOSE: In patients with pulmonary venoocclusive disease (PVOD), intravenous prostacyclin therapy has been reported to cause life-threatening pulmonary edema and even death. We report 3 patients with presumed PVOD treated successfully with the inhaled prostacyclin, iloprost.
METHODS: Retrospective analysis of clnical characteristics, adverse events, and efficacy was performed in 3 patients meeting clnical criteria for PVOD. These criteria included “classic” findings on Chest CT, severe hypoxemia without intracardiac shunting, markedly reduced DLCO in the absence of interstitial lung disease, and worsening clinical status or gas exchange on oral bosentan.
RESULTS: All 3 patients were on sildenafil at the time of iloprost initiation. No adverse events were noted following iloprost and during follow-up of 12, 14, and 34 weeks.
CONCLUSION: In 3 patients with PVOD already on sildenafil, inhaled iloprost was well tolerated, did not lead to pulmonary edema, and was associated with improved symptoms.
CLINICAL IMPLICATIONS: Inhaled iloprost may represent a beneficial therapy in PVOD, without the risk associated with intravenous prostanoids.
DISCLOSURE: Rajeev Saggar, Grant monies (from industry related sources) 30000; Consultant fee, speaker bureau, advisory committee, etc. 30000.