Recommendations

Document Overview

Design and created by Guideline Central in participation with the American College of Chest Physicians.

Therapy For Pulmonary Arterial Hypertension In Adults

American College of Chest Physicians

Publication Date: Jan 1, 2019

Page Last Updated: May 5, 2026

CHEST Grading System

GRADE	Grade of Recommendation		Beneﬁt vs Risk and Burdens	Methodologic Strength of Supporting Evidence	Implications
S-H	Strong recommendation, high-quality evidence		Beneﬁts clearly outweigh risk and burdens, or vice versa.	We are very conﬁdent that the true effect lies close to that of the estimate of the effect.	Recommendation can apply to most patients in most circumstances. Further research is very unlikely to change our conﬁdence in the estimate of effect.
S-M	Strong recommendation, moderate-quality evidence		Beneﬁts clearly outweigh risk and burdens, or vice versa.	We are moderately conﬁdent in the effect estimate: the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different.	Recommendation can apply to most patients in most circumstances. Higher-quality research may well have an important impact on our conﬁdence in the estimate of effect and may change the estimate.
S-L	Strong recommendation, low-quality evidence		Beneﬁts clearly outweigh risk and burdens, or vice versa.	Our conﬁdence in the effect estimate is limited: the true effect may be substantially different from the estimate of the effect.	Recommendation can apply to most patients in many circumstances. Higher-quality research is likely to have an important impact on our conﬁdence in the estimate of effect and may well change the estimate.
S-VL	Strong recommendation, very low-quality evidence		Beneﬁts clearly outweigh risk and burdens, or vice versa.	We have very little conﬁdence in the effect estimate: the true effect is likely to be substantially different from the estimate of effect	Recommendation can apply to most patients in many circumstances. Higher-quality research is likely to have an important impact on our conﬁdence in the estimate of effect and may well change the estimate.
W-H	Weak (conditional) recommendation, high-quality evidence		Beneﬁts closely balanced with risks and burden.	We are very conﬁdent that the true effect lies close to that of the estimate of the effect.	The best action may differ depending on circumstances or patients’ or societal values. Further research is very unlikely to change our conﬁdence in the estimate of effect.
W-M	Weak (conditional) recommendation, moderate-quality evidence		Beneﬁts closely balanced with risks and burden.	We are moderately conﬁdent in the effect estimate: the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different	Best action may differ depending on circumstances or patients’ or societal values. Higher-quality research may well have an important impact on our conﬁdence in the estimate of effect and may change the estimate.
W-L	Weak (conditional) recommendation, low-quality evidence		Uncertainty in the estimates of beneﬁts, risks, and burden; beneﬁts, risk, and burden may be closely balanced.	Our conﬁdence in the effect estimate is limited: the true effect may be substantially different from the estimate of the effect.	Other alternatives may be equally reasonable. Higher-quality research is likely to have an important impact on our conﬁdence in the estimate of effect and may well change the estimate.
W-VL	Weak (conditional) recommendation, very-low quality evidence		Uncertainty in the estimates of beneﬁts, risks, and burden; beneﬁts, risk, and burden may be closely balanced.	We have very little conﬁdence in the effect estimate: the true effect is likely to be substantially different from the estimate of effect.	Other alternatives may be equally reasonable. Higher-quality research is likely to have an important impact on our conﬁdence in the estimate of effect and may well change the estimate.
	Ungraded consensus-based suggestions
U-CBS	Ungraded Consensus-Based Statement	Uncertainty because of lack of evidence but expert opinion that beneﬁts outweigh risk and burdens or vice versa.		Insufﬁcient evidence for a graded recommendation.	Future research may well have an important impact on our conﬁdence in the estimate of effect and may change the estimate.

Document Overview

Document Title: Therapy For Pulmonary Arterial Hypertension In Adults

Authoring Society: American College of Chest Physicians

Document Publication Date: Jan 1, 2019

Page Last Reviewed/Updated: May 5, 2026

Document Type: Guideline

Country of Publication: United States

Full Text Freely Available: Yes

Full Text Guideline: journal.chestnet.org/article/S0012-3692(19)30002-9/fulltext

Source Citation: DOI: https://doi.org/10.1016/j.chest.2018.11.030

Supplemental Implementation Resources

Algorithm

Document Scope, Criteria, and Use Cases

Document Objectives: Pulmonary arterial hypertension (PAH) carries a poor prognosis if not promptly diagnosed and appropriately treated. The development and approval of 14 medications over the last several decades have led to a rapidly evolving approach to therapy, and have necessitated periodic updating of evidence-based treatment guidelines. This guideline statement, which now includes a visual algorithm to enhance its clinical utility, represents the fourth iteration of the American College of Chest Physicians Guideline and Expert Panel Report on Pharmacotherapy for PAH.

Scope: Diagnosis, Management, Treatment

Diseases/Conditions (MeSH)

D006976 - Hypertension, Pulmonary

D000081029 - Pulmonary Arterial Hypertension

D006976 - Hypertension, Pulmonary

D000081029 - Pulmonary Arterial Hypertension

Keywords: hypertension, pulmonary arterial hypertension

Target Patient Population: Adult patients with pulmonary arterial hypertension

Inclusion Criteria: Male, Female, Adolescent, Adult, Older Adult

Health Care Settings: Ambulatory, Hospital, Outpatient

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant

Recommendation Development Processes & Methodology

Supplemental Methodology Resources: Data Supplement Data Supplement

Number of Source Documents: 52

Literature Search Start Date: Sunday, January 1, 2012

Literature Search End Date: Friday, July 1, 2016

Includes peer/external review process?: Yes

Includes public comment process?: No

Methodologist involvement?: Yes

Patient involvement?: No

Includes multi-disciplinary group?: Yes

Includes systematic review?: Yes

Grades quality of strength of evidence?: Yes

Grades quality of strength of recommendation?: Yes

Discloses funding source?: Yes

Discloses conflicts of interest?: Yes

Includes benefits/harms analysis with recommendations?: No

Therapy For Pulmonary Arterial Hypertension In Adults

Recommendations

Treatment Naive PAH Patients Without Symptoms (WHO FC I) and Patients at Increased Risk for the Development of PAH

Symptomatic Patients With PAH

Vasoreactivity Testing and Use of Calcium Channel Blockers (CCBs)

PAH-Speciﬁc Pharmacotherapies

Patients With WHO FC II Symptoms

Patients With WHO FC III Symptoms

Patients With WHO FC IV Symptoms

PAH Patients on Established PAH-Speciﬁc Therapy

Combination Studies of Endothelin Receptor Antagonists and Phosphodiesterase Inhibitors

Palliative Care and Supportive Therapies

Preventive Care

Specific Patient Situations

Pregnancy

Altitude and Air Travel

Surgery

CHEST Grading System

Document Overview

Supplemental Implementation Resources

Document Scope, Criteria, and Use Cases

Recommendation Development Processes & Methodology