PURPOSE: Intravenous (IV) epoprostenol and IV/subcutaneous treprostinil are accepted therapies for severe pulmonary arterial hypertension (PAH), however adverse effects including infection and site pain limit their use in some patients. Inhaled iloprost may be an alternative therapeutic option for prostacyclin therapy but there is limited published data regarding its safety and efficacy after the transition from parenteral prostanoid therapy.
METHODS: Records of patients transitioned from parenteral prostanoid therapy to inhaled iloprost (n=5) due to adverse effects were reviewed for survival , functional status and hemodynamic data via right heart catheterization (RHC, if available) to determine early and midterm outcomes.
RESULTS: Pulmonary arterial (PA) mean pressure measured via right heart catheterization while patients were receiving parenteral prostanoid therapy ranged from 47-90 mm Hg. Diagnoses were idiopathic PAH (iPAH; n=2), Portopulmonary hypertension(n=1), Eisenmenger sydrome(n=2); Prostanoids included IV epoprostenol (n=2) and subcutaneous treprostinil (n=3). Reasons for change in therapy included infection (n=2), site pain (n=2) and nausea/vomiting (n=1). Outcomes include one patient (iPAH) who decided against inhaled therapy and converted from flolan to treprostinil, another (iPAH) remained functionally stable on inhaled iloprost at 2 months post-transition with stable pulmonary pressures by RHC, but subsequently died due to withdrawl of care after requiring invasive mechanical ventilation for respiratory failure. The remaining three patients are alive with stable function and stable hemodynamic measurements with a mean of 431 (range 210-706) days following the transition to inhaled iloprost.
CONCLUSION: Of the 4 patients fully transitioned to inhaled iloprost from parenteral prostanoid therapy, all were functionally stable in the short-term and 3 of 4 have experienced continuing stability and no deterioration in WHO functional status for 7-23 months.
CLINICAL IMPLICATIONS: The transition from parenteral prostanoid therapy to inhaled iloprost was well tolerated from a hemodynamic and functional standpoint in a pilot group of patients with pulmonary arterial hypertension of varying underlying etiologies. Inhaled iloprost may provide a reasonable therapeutic alternative for selected patients with moderate to severe PAH.
DISCLOSURE: Sean Studer, No Product/Research Disclosure Information; Consultant fee, speaker bureau, advisory committee, etc. Consultant for Actelion