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Characterization of Patients Enrolled in RESPIRE, the Registry to Prospectively Evaluate Use of Ventavis® in Patients with Pulmonary Arterial Hypertension

Curt Daniels


Sean M. Studer


Franck Rahaghi

J McConnell

Jean Elwing

J. S. Sager

R Zolty

S Petersen

S Short

D. Villanueva

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Conference: 2013 PH Professional Network Symposium

Release Date: 09.28.2013

Presentation Type: Abstracts

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*Characterization of Patients Enrolled in RESPIRE, the Registry to Prospectively Evaluate Use of Ventavis® in Patients with Pulmonary Arterial Hypertension


C. Daniels, MD1; S. Studer, MD, MS2; F. Rahaghi, MD3; J. McConnell, MD4; J. Elwing, MD, FCCP5; J. Sager, MD, MSCE, FCCP6; R. Zolty,MD7; S. Petersen, MS8; S. Short, MPH9; D. Villanueva, MD8; W. Benton, PharmD8; N. Kim, MD10

1Columbus Ohio Adult Congenital Heart Disease (COACH) Program, Nationwide Children’s Hospital and The Ohio State University, Columbus, Ohio;2Newark Beth Israel Medical Center, Newark, N.J.; 3Cleveland Clinic Florida, Weston, Fla.; 4Kentuckiana Pulmonary Associates, Louisville, Ky.;5University of Cincinnati, Cincinnati, Ohio; 6Santa Barbara Pulmonary Consultants LLC, Santa Barbara, Calif.; 7Montefiore Medical Center, Bronx,N.Y.; 8Actelion Pharmaceuticals US, Inc., South San Francisco, Calif.; 9ICON Late Phase & Outcomes Research, San Francisco, Calif.; 10University ofCalifornia San Diego, La Jolla, Calif.


PURPOSE: To characterize patients enrolled in RESPIRE (Registry To Prospectively Evaluate Use Of Ventavis® In Patients With Pulmonary ArterialHypertension), a registry initiated to assess the association between patient characteristics at study entry and adherence to inhaled iloprost, tomeasure adherence under different interventions, and to examine relationships between these interventions and inhaled iloprost persistence.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a chronic, progressive disease characterized by increased pulmonary artery pressureand vascular resistance, leading to right heart failure and death. Underlying physiological impairments are targeted by current therapies includingthe prostacyclin analogue Ventavis® (iloprost) inhalation solution, indicated for treatment of PAH, NYHA functional class III-IV.

METHOD: RESPIRE is an ongoing, 32-center, observational, US-based study initiated on March 30, 2009, to follow patients with PAH over a 2-yearperiod who had been receiving inhaled iloprost for ≥3 months at enrollment. Data were collected via patient interviews, medical record review,and I-neb Adaptive Aerosol Delivery (AAD) device download. Medications and inhaled iloprost adherence data were collected at 1 month andquarterly post-enrollment. Adherence was defined as the number of days using inhaled iloprost divided by the number of days eligible. Persistence was determined from enrollment to first occurrence of inhaled iloprost discontinuation for reasons other than death, death, withdrawal of consent, loss to follow-up, or end of study, whichever occurred first.

FINDINGS: Of 148 eligible patients, 38 (26%) used iloprost 10 μg/mL (5-μg dose) and 105 (71%) iloprost 20 μg/mL (5-μg dose) at enrollment(Table); 5 (3%) patients on iloprost 10 μg/mL (2.5-μg dose) were excluded from the analytic cohort (N=143). At enrollment, patients with PAHwere (mean ± SD) aged 63 ± 13 years, diagnosed 47 ±4 1 months prior, and receiving inhaled iloprost for 16 ± 16 months. The majority of theanalytic cohort was functional class III and IV (50% and 4%, respectively), female (80%), white (70%), and had idiopathic PAH (65%). Fifty-eight(41%) patients met discontinuation criteria at 7 ± 5 months, primarily due to discontinuation of inhaled iloprost (52%). Post-enrollment, patientadherence was 81 ± 26%, with 6 ± 14% incomplete treatments, 4 ± 2 treatments/day, and 7 ± 4 minutes inhalation time/treatment. Fiftyseven(40%) patients completed the 12-month assessment, with 1-year freedom from inhaled iloprost discontinuation from registry enrollmentestimates ± SE for the total cohort, 10-μg/mL subgroup, and 20-μg/mL subgroup of 59 ± 5%, 60 ± 9%, and 59 ± 6%, respectively, and 1-yearsurvival from registry enrollment of 90 ± 3%, 88 ± 6%, and 90 ± 4%, respectively (Figure 1A and B).

IMPLICATIONS: Data from the RESPIRE registry provide valuable information about the characteristics and adherence of patients with PAHreceiving chronic inhaled iloprost therapy.

Table 

2013 Symposium Abstract: Characterization Table1

APAH, associated with PAH; CHD, congenital heart disease; CTD, connective tissue disease; IPAH, idiopathic PAH; PAH, pulmonary arterialhypertension; PoPH, portopulmonary hypertension; SD, standard deviation.a Includes the following ethnicities: Native American or Native Alaskan, Asian or Pacific Islander, Other, and Unknown.bBased on the date of PAH diagnosis listed on the case report form.

2013 Symposium Abstract: Characterization Table 2

APAH, associated with PAH; CHD, congenital heart disease; CTD, connective tissue disease; IPAH, idiopathic PAH; PAH, pulmonary arterialhypertension; PoPH, portopulmonary hypertension; SD, standard deviation.


*This study was sponsored by Actelion Pharmaceuticals US, Inc.