Treatment of pulmonary arterial hypertension algorithm

If no improvement or patients progress, therapy should be escalated according to WHO functional symptoms. For patients on medications for other conditions, specific drug interactions and management suggestions may be determined by using the drug interactions program included with UpToDate.

INH: inhaled; IV: intravenous; SC: subcutaneous; WHO: World Health Organization.

* Acute vasoreactivity testing is typically indicated in patients with WHO class I, II, and III symptoms who have idiopathic, hereditary, or drug/toxin-induced PAH. Contraindications include patients with WHO class IV symptoms, low systemic blood pressure, and low cardiac index.

¶ Endothelin receptor antagonist-phosphodiesterase-5 inhibitor combination is preferred by most experts. Combining phosphodiesterase-5 inhibitors and guanylate cyclase stimulants (riociguat) should be avoided due to the high risk of hypotension.

Δ Options for agents include ambrisentan, bosentan, macitentan, sildenafil, tadalafil, or riociguat. Riociguat is best studied in patients with chronic thromboembolic pulmonary hypertension. Sotatercept may be considered as an addon agent to standard therapy.

◊ These agents are not approved for this use by regulatory agencies.

§ Some experts use initial combination therapy with a prostanoid and a phosphodiesterase-5 inhibitor.

Original figure modified for this publication. Reproduced from: Barst RJ, Gibbs US, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54:S78. Illustration used with the permission of Elsevier Inc. All rights reserved.

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Treatment of pulmonary arterial hypertension algorithm