PAH Therapy Guidelines Updated by CHEST Expert Panel

Doctor comforting patient
Doctor comforting patient
A panel of experts and organizational representatives updated the 2014 CHEST guidelines for pulmonary arterial hypertension therapy.

A panel of experts and organizational representatives from the Pulmonary Hypertension Association, American Thoracic Society, and American Heart Association have updated the 2014 American College of Chest Physicians Guideline and Expert Panel Report on Pharmacotherapy for pulmonary arterial hypertension (PAH). The update was published in CHEST.

Studies published after the completion of the 2014 guideline were reviewed by a guideline panel, including 3 cochairs, 3 panelists, representatives from national related organizations, and a patient representative. The population, intervention, comparator, outcome (PICO) format was used to develop key clinical questions included in the guideline. The panel assessed the quality of the evidence for critical or important outcomes of interest with the Grading of Recommendations Assessment, Development and Evaluation approach. In addition, a modified Delphi technique was used to reach a consensus regarding graded recommendations and ungraded consensus-based statements.

Of the 300 studies published after the 2014 guidelines, only 4 studies met the inclusion criteria, all of which discussed combination therapies used for PAH. Based on the moderate quality of evidence from these studies, the panel provided a weak recommendation for initial combination therapy with ambrisentan and tadalafil in treatment-naive patients with PAH with World Health Organization functional class II and III to improve 6-minute walking distance. Based on low-quality evidence, the panel also recommended adding tadalafil to ambrisentan background therapy to improve 6-minute walking distance in patients with stable or symptomatic PAH.

In addition, the new update provided 2 ungraded consensus-based statements on palliative care. First, the guideline suggested incorporating palliative care services in a treatment protocol for patients with PAH. Second, the update recommended the use of supervised exercise as part of integrated care in patients with PAH.

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“Importantly, we continue to suggest early referral to expert centers and collaborative care using sound clinical judgment,” the panel concluded. “Looking to the future, we strongly encourage the translation of basic discovery into safe and effective new therapies through the conduct of well-designed clinical trials.”


Klinger JR, Elliott G, Levine DJ, et al. Therapy for pulmonary arterial hypertension in adults 2018: update of the CHEST guideline and expert panel report [published online January 17, 2019]. CHEST. doi:10.1016/j.chest.2018.11.030