@article{fa5e429c2f3e4a18b2ec06c7393435c8,
title = "Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report",
abstract = "OBJECTIVE: Choices of pharmacologic therapies for pulmonary arterial hypertension (PAH) are ideally guided by high-level evidence. The objective of this guideline is to provide clinicians advice regarding pharmacologic therapy for adult patients with PAH as informed by available evidence. METHODS: This guideline was based on systematic reviews of English language evidence published between 1990 and November 2013, identified using the MEDLINE and Cochrane Library databases. The strength of available evidence was graded using the Grades of Recommendations, Assessment, Development, and Evaluation methodology. Guideline recommendations, or consensus statements when available evidence was insufficient to support recommendations, were developed using a modified Delphi technique to achieve consensus. RESULTS: Available evidence is limited in its ability to support high-level recommendations. Therefore, we draft ed consensus statements to address many clinical questions regarding pharmacotherapy for patients with PAH. A total of 79 recommendations or consensus statements were adopted and graded. CONCLUSIONS: Clinical decisions regarding pharmacotherapy for PAH should be guided by high-level recommendations when sufficient evidence is available. Absent higher level evidence, consensus statements based upon available information must be used. Further studies are needed to address the gaps in available knowledge regarding optimal pharmacotherapy for PAH.",
author = "Taichman, {Darren B.} and Joe Ornelas and Lorinda Chung and Klinger, {James R.} and Sandra Lewis and Jess Mandel and Palevsky, {Harold I.} and Stuart Rich and Namita Sood and Rosenzweig, {Erika B.} and Trow, {Terence K.} and Rex Yung and Elliott, {C. Gregory} and Badesch, {David B.}",
note = "Funding Information: FUNDING/SUPPORT: This study was funded in total by internal funds from the American College of Chest Physicians. Funding Information: Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Until 2009, Dr Taichman was an employee of the University of Pennsylvania, which received research grant support from Actelion Pharmaceuticals Ltd for participation in the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry). He received honoria for CME talks sponsored by the Pulmonary Hypertension Association. Since 2009, he has been an employee of the American College of Physicians. Dr Chung receives grant funding from the Scleroderma Research Foundation. She has received compensation for clinical trial patient enrollment from Gilead; Actelion Pharmaceuticals Ltd; United Therapeutics Corp; Pfizer Inc; MedImmune LLC; Genentech, Inc; and InterMune. She has participated in speaking activities for Actelion Pharmaceuticals Ltd and Gilead and has served on the Advisory Board for Gilead. Dr Lewis is an officer in an institution that probably has a financial relationship with a commercial entity having an interest in the subject of this manuscript. However, the time of working on this manuscript did not overlap with the time she has been employed by this company. She also makes public statements on guideline methodology. Dr Klinger has served as a site investigator for numerous clinical studies in pulmonary hypertension and in numerous pharmacologic, industry-sponsored studies in pulmonary hypertension. Dr Klinger has served as a consultant for Bayer and United Therapeutics Corp. He has served on the steering committee and adjudication committee for industry-sponsored clinical trials. He has received grant support from the National Institutes of Health (NIH) for enrollment of patients in clinical registries. Dr Mandel receives royalties as an author and editor from Elsevier BV and Wolters-Kluwer. Dr Palevsky has within the past 3 years served as a consultant for Actelion Pharmaceuticals Ltd, Bayer AG, Gilead, and United Therapeutics Corp, and has served on data and safety monitoring boards for Aires Therapeutics and Pfizer Inc. He has also served as a grant reviewer for the Entelligence PAH Young Investigators Award Program and has given continuing medical education and other PAH lectures. Dr Rich has received money for speaker's fees from GlaxoSmithKline. Dr Rich has no other intellectual or financial conflicts to disclose. While he had worked in industry in the past, this ended in 2006. Dr Sood has received pharmaceutical grant money for research products and serves as a consultant for advisory board meetings for Actelion Pharmaceuticals Ltd, Bayer AG, Gilead, and United Therapeutics Corp. Dr Rosenzweig has received honoraria from Actelion, Gilead Science, and United Therapeutics as an advisor on Scientific Advisory Board Panels and Ikaria for a study oversight committee in the past three years. Dr Trow has in the distant past served as a consultant for Bayer AG, Actelion Pharmaceuticals Ltd, Gilead, and United Therapeutics Corp. He also used to serve on Speaker's Bureaus for Actelion Pharmaceuticals Ltd, Gilead, and United Therapeutics Corp. No such talks have been given in the 2.5 years prior to this publication. In addition, Dr Trow has served as the primary investigator on the COMPASS (Effects of Combination of Bosentan and Sildenafil vs Sildenafil Monotherapy on Morbidity and Mortality in Symptomatic Patients with PAH) 2 trial and Registry to Prospectively Evaluate Use of Ventavis in Patients With PAH (RESPIRE Registry), which have now finished. Dr Elliott is employed by Intermountain Healthcare (IHC Health Service, Inc) and IHC Health Services, Inc has received compensation trials (on which he is the principal investigator) from Actelion Pharmaceuticals Ltd, Bayer AG, GeNo, Gilead, and United Therapeutics Corp. Dr Elliott serves on the End-Point Adjudication Committee for a study sponsored by Lung LLC. Both he and IHC Health Services received compensation for his service on the End-Point Adjudication Committee. He has received travel and reimbursement for meetings he attended sponsored by Bayer AG, Lung LLC, and Ikaria. He serves as a consultant to Bayer Pharmaceuticals. He received honoria for serving on the REVEAL Steering Committee, which was supported by CoTherix/Actelion. He serves on the board of directors for the Pulmonary Hypertension Association, served on the American College of Chest Physicians Consensus Guidelines Committee for Pulmonary Arterial Hypertension, and is an advisor for the Scientific Leadership Council of the Pulmonary Hypertension Associations. Dr Badesch has received honoraria for service on steering committees or advisory boards (or as a consultant) to the following companies working in the area of pulmonary hypertension: Actelion Pharmaceuticals Ltd/CoTherix; Gilead; Pfizer Inc; United Therapeutics Corp/Lung Rx; Bayer AG; Ikaria, Inc; and Arena Pharmaceuticals, Inc. He has received grant support for clinical studies from Actelion Pharmaceuticals Ltd/CoTherix; Gilead; Pfizer Inc; United Therapeutics Corp/Lung Rx; Bayer AG; Novartis AG; Ikaria, Inc; and Reata Pharmaceuticals Inc. He provided information pertinent to a legal matter for Actelion Pharmaceuticals, Inc. Mr Ornelas and Dr Yung have reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article. Funding Information: Role of sponsors: This study was funded in total by internal funds from the American College of Chest Physicians. Copyright: Copyright 2018 Elsevier B.V., All rights reserved.",
year = "2014",
month = aug,
doi = "10.1378/chest.14-0793",
language = "English (US)",
volume = "146",
pages = "449--475",
journal = "Chest",
issn = "0012-3692",
publisher = "American College of Chest Physicians",
number = "2",
}