PURPOSE: Inhaled iloprost is a recommended therapy for patients with moderate to severe pulmonary arterial hypertension (PAH). A better understanding of the long-term clinical outcomes of these patients is important in guiding future treatment decisions. Here we aim to describe the clinical course of PAH in the setting of inhaled iloprost therapy for a duration of at least 1 year.
METHODS: Patients with PAH aged ≥18 years who initiated therapy with inhaled iloprost between 30 December 2004 and 31 December 2007, received inhaled iloprost for at least 1 year, and had 6-minute walk test distance (6-MWD) at enrollment and at least one follow-up were eligible for this retrospective chart review. Patients who halted therapy for >28 days for any reason were excluded. Outcomes investigated included change in 6-MWD and PAH-specific therapies at baseline and after 1 year (inhaled iloprost remaining a constant therapy).
RESULTS: The charts of 36 patients from 12 centers were reviewed. Mean age at baseline was 54 ± 15 years; 83% were female. 1, 5, 16, and 2 patients were functional class (FC) I, II, III, and IV, respectively. FC was unknown for 12 patients. Baseline 6-MWD was 309 ± 146 m. Mean duration of PAH was 2.1 ± 2.2 years. 25% of patients were initiated on inhaled iloprost as monotherapy; 75% were on combination therapy. Over the 1-year period of iloprost therapy, other PAH-specific therapy was added in 25%, remained constant in 50%, changed in 19%, and reduced in 6% of patients. 6-MWD increased 34 ± 79 m (P = 0.013) from Day 1 to 365. 6-MWD was maintained or improved in 26/36 patients. 12 patients experienced an increase of >60 m.
CONCLUSIONS: The majority of patients on inhaled iloprost ≥1 year maintain or improve their 6-MWD.
CLINICAL IMPLICATIONS: Long-term responders to inhaled iloprost maintain or improve their walk distance. Results need to be interpreted with caution given the retrospective nature of this study and the fact that patients who discontinued iloprost therapy within 1 year were not included.
DISCLOSURE: John McConnell: Consultant fee, speaker bureau, advisory committee, etc.: Dr. McConnell has received compensation for speaking engagements from United Therapeutics, Actelion, Gilead, and Boehringer-Ingelheim, and has received compensation for advisory board for Actelion.
Steven Nathan: Consultant fee, speaker bureau, advisory committee, etc.: Consultant and speakers bureau for Actelion, Gilead and United Therapeutics; consultant for GeNO and Bayer, Grant monies (from industry related sources): Research funding from Actelion, Gilead and United Therapeutics
Kimberly Hobbs: Consultant fee, speaker bureau, advisory committee, etc.: Kim Hobbs has received an honorarium from Actelion for advisory board
Desilu Villanueva: Employee: Employee of Actelion Pharmaceuticals US, Inc.
Brian Hartline: Employee: Employee of Actelion Pharmaceuticals US, Inc.
Robert Frantz: Other: Dr Frantz has received support for traveling to REVEAL meetings from Actelion , Consultant fee, speaker bureau, advisory committee, etc.: Dr Frantz’s institution has received consulting fees or honorarium, and has been in consultancy relationships with Actelion, Gilead, and United Therapeutics, Grant monies (from industry related sources): Dr Frantz’s institution has received grants or has grants pending from Actelion, Gilead, and United Therapeutics
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