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Pharmacologic Therapy for Pulmonary Arterial Hypertension in Adults
Open accessAbstract
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 drafted 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.
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“Given that most efficacious therapies for the treatment of PH involve targeting of NO signaling[48,49], it is clear that pulmonary vascular health is intimately tied to effective NO bioactivity.”
From Enterosalivary nitrate metabolism and the microbiome: Intersection of microbial metabolism, nitric oxide and diet in cardiac and pulmonary vascular health (2017) · cites this paper as [48]
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Metadata
- Authors
- Taichman, D. B., Ornelas, J., Chung, L., Klinger, J. R., Lewis, S., Mandel, J., Palevsky, H. I., Rich, S., Sood, N., Rosenzweig, E. B., Trow, T. K., Yung, R., Elliott, C. G., & Badesch, D. B.
- Affiliations
- University of Pennsylvania (US)
- American College of Chest Physicians (US)
- VA Palo Alto Health Care System (US)
- Brown University (US)
- University of California San Diego (US)
- University of Chicago (US)
- The Ohio State University (US)
- Columbia University Irving Medical Center (US)
- Yale University (US)
- Johns Hopkins University (US)
- University of Utah (US)
- University of Colorado Denver (US)
- Corresponding author
- Ornelas, Joe
- ORCID
- Venue
- Chest
- Publisher
- Elsevier BV
- Published
- 2014-08
- Volume
- 146
- Issue
- 2
- Pages
- 449-475
- ISSN
- 0012-3692 (ISSN-L)
- PMC
- PMC4137591
- OpenAlex
- W2157947374
- Cited by
- 256
- References
- 104
- Citations by year
- 2026: 6 · 2025: 7 · 2024: 6 · 2023: 5 · 2022: 13 · 2021: 18 · 2020: 14 · 2019: 25 · 2018: 29 · 2017: 43 · 2016: 40 · 2015: 43 · 2014: 5
- FWCI
- 7.47
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- CHEST Journal (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Genetics (clinical) › Vascular Anomalies and Treatments
- Health Sciences › Medicine › Internal Medicine › Venous Thromboembolism Diagnosis and Management
- Keywords
- pulmonary arterial hypertension, pharmacotherapy, adult patients, antihypertensive drugs, consensus statement, treatment guidelines, level of evidence
- MeSH terms
- Adult, Antihypertensive Agents, Delphi Technique, Humans, Hypertension, Pulmonary, Practice Guidelines as Topic, Consensus, Familial Primary Pulmonary Hypertension
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