Pulmonary Vascular Disease: PAH |

Expert Consensus on the Prescribing Practice and Management of Adverse Events Associated With the Treatment of Patients Taking Macitentan for PAH: A Delphi Consensus Study FREE TO VIEW

Franck Rahaghi, MD; Hassan Alnuaimat, MD; Rana Awdish, MD; Vijay Balasubramanian, MD; Robert Bourge, MD; Charles Burger, MD; John Butler, MD; C. Gregory Cauthen, MD; Murali Chakinala, MD; Bennett deBoisblanc, MD; Michael Eggert, MD; Peter Engel, MD; Jeremy Feldman, MD; J. Wesley McConnell, MD; Myung Park, MD; Jeffrey Sager, MD; Namita Sood, MD; Harold Palevsky, MD
Author and Funding Information

Cleveland Clinic, Weston, FL

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1148A. doi:10.1016/j.chest.2016.08.1258
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SESSION TYPE: Original Investigation Slide

PRESENTED ON: Sunday, October 23, 2016 at 01:30 PM - 03:00 PM

PURPOSE: Pulmonary arterial hypertension (PAH) is a rare disease characterized by elevated pulmonary vascular resistance. Macitentan is an endothelin receptor antagonist indicated for the long-term treatment of PAH. This study presents consensus recommendations for managing macitentan and associated adverse events in PAH patients.

METHODS: A three stage Delphi method was designed to elicit feedback on the prescribing practice and management of adverse events associated with the treatment of patients taking macitentan. The expert panel included physicians with experience treating PAH (18.1 ± 7.6 years, 247 ± 255 patients) and prescribing macitentan (70 ± 60 patients). Survey one queried panelists regarding therapeutic approaches based on the prescribing information and pivotal phase III clinical trial results. Surveys two and three had panelists rank their agreement/disagreement (+3 to -3, respectively) with recommendations and arrive at a consensus (≥75%).

RESULTS: Consensus statements include how to manage treatment interruptions, dual or triple therapy use, and prescribing practices based on patient functional class. Expert panel recommendations favored using macitentan as a single agent or part of a combination therapy, symptomatic care to treat bronchitis or nasopharyngitis, and adding a prostanoid to treat right ventricular failure (see below). Topline summary of major consensus (≥75%) recommendations Categories not covered by publications or prescribing information To Reduce PAH Hospitalizations (mean ± std dev) • Prescribe for Functional Class II as a single agent (1.5 ± 1.3) • Prescribe for Functional Class II - III as part of a combination (2.2 ± 0.8) • Prescribe for Functional Class IV as a single agent (-2.5 ± 0.6) First-line Adverse Events Frequency & Management (mean ± std dev) • Anemia: Monitor with CBC every 3 months (2.0 ± 0.8) • Bronchitis: Symptomatic care/therapy (2.2 ± 0.4), pneumonia and influenza vaccination (1.8 ± 1.8) • Cough: Symptomatic care/therapy (2.0 ± 1.2), benzonatate capsules (1.3 ± 1.1) • Dizziness: Evaluate status of PAH (2.6 ± 0.5) • Dyspnea: Evaluate status of PAH (2.7 ± 0.5) • Headache: Acetaminophen (2.1 ± 0.6) • Influenza: Vaccinate (annually) (2.7 ± 0.5) • Nasopharyngitis/Pharyngitis: Symptomatic care/therapy (2.3 ± 0.4), nasal spray (eg, fluticasone, azelastine, ipratropium nasal inhalers) (1.9 ± 0.9) • Peripheral Edema: Educate patient on diet, including salt and fluid intake (2.8 ± 0.4) • Right Ventricular Failure: Add/increase prostanoid (2.1 ± 0.7), repeat right heart catheterization and add prostacyclin (2.1 ± 0.8), maximize PAH meds/diuretics (2.1 ± 0.9), add parenteral PAH therapy (2.1 ± 1.0) • Upper Respiratory Tract Infection: Symptomatic care/treatment (2.3 ± 0.8) • General Recommendations on Administration: Educate patient on dietary, fluid and sodium restrictions, and assess adherence (2.6 ± 0.5)

CONCLUSIONS: A set of consensus opinion statements regarding the prescription and use of macitentan in PAH patients have been created for use until there is evidence-based data from further studies.

CLINICAL IMPLICATIONS: Absent evidence-based data, these consensus statements provide direction on the practical management of medications for rare disease patients.

DISCLOSURE: Franck Rahaghi: Grant monies (from industry related sources): Unrestricted Educational Grant from Actelion Pharmaceuticals The following authors have nothing to disclose: Hassan Alnuaimat, Rana Awdish, Vijay Balasubramanian, Robert Bourge, Charles Burger, John Butler, C. Gregory Cauthen, Murali Chakinala, Bennett deBoisblanc, Michael Eggert, Peter Engel, Jeremy Feldman, J. Wesley McConnell, Myung Park, Jeffrey Sager, Namita Sood, Harold Palevsky

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