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Cited by 499

The DOI matches its Crossref record; 70 of the 70 references it cites were found in a registry or an index.

Updated Evidence-Based Treatment Algorithm in Pulmonary Arterial Hypertension

Open access
Barst, Robyn J., Gibbs, J. Simon R., Ghofrani, Hossein A., Hoeper, Marius M., McLaughlin, Vallerie V., Rubin, Lewis J., Sitbon, Olivier, Tapson, Victor F., Galiè, Nazzareno·2009·Journal of the American College of Cardiology
DOI 10.1016/j.jacc.2009.04.017
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Abstract

Uncontrolled and controlled clinical trials with different compounds and procedures are reviewed to define the risk-benefit profiles for therapeutic options in pulmonary arterial hypertension (PAH). A grading system for the level of evidence of treatments based on the controlled clinical trials performed with each compound is used to propose an evidence-based treatment algorithm. The algorithm includes drugs approved by regulatory agencies for the treatment of PAH and/or drugs available for other indications. The different treatments have been evaluated mainly in idiopathic PAH, heritable PAH, and in PAH associated with the scleroderma spectrum of diseases or with anorexigen use. Extrapolation of these recommendations to other PAH subgroups should be done with caution. Oral anticoagulation is proposed for most patients; diuretic treatment and supplemental oxygen are indicated in cases of fluid retention and hypoxemia, respectively. High doses of calcium channel blockers are indicated only in the minority of patients who respond to acute vasoreactivity testing. Nonresponders to acute vasoreactivity testing, or responders who remain in World Health Organization (WHO) functional class III, should be considered candidates for treatment with either an oral phosphodiesterase-5 inhibitor or an oral endothelin-receptor antagonist. Continuous intravenous administration of epoprostenol remains the treatment of choice in WHO functional class IV patients. Combination therapy is recommended for patients treated with PAH monotherapy who remain in New York Heart Association functional class III. Atrial septostomy and lung transplantation are indicated for refractory patients or where medical treatment is unavailable.

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Metadata

Authors
Barst, R. J., Gibbs, J. S. R., Ghofrani, H. A., Hoeper, M. M., McLaughlin, V. V., Rubin, L. J., Sitbon, O., Tapson, V. F., & Galiè, N.
Venue
Journal of the American College of Cardiology
Publisher
Elsevier BV
Published
2009-06
Volume
54
Issue
1
Pages
S78-S84
ISSN
0735-1097 (ISSN-L)
DOI
10.1016/j.jacc.2009.04.017
OpenAlex
W2142743112
Cited by
499
References
70
FWCI
64.03
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: Updated Evidence-Based Treatment Algorithm in Pulmonary Arterial Hypertension. https://app.cochat.ai/papers/10.1016/j.jacc.2009.04.017
The paper
Barst, R. J., Gibbs, J. S. R., Ghofrani, H. A., Hoeper, M. M., McLaughlin, V. V., Rubin, L. J., Sitbon, O., Tapson, V. F., & Galiè, N. (2009). Updated Evidence-Based Treatment Algorithm in Pulmonary Arterial Hypertension. Journal of the American College of Cardiology. https://doi.org/10.1016/j.jacc.2009.04.017

Data sources

Bibliographic data: Semantic Scholar, OpenAlex, Crossref.

Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.