Vnitr Lek 2026, 72(2):86-91 | DOI: 10.36290/vnl.2026.019

Recent advances in the pharmacotherapy of pulmonary arterial hypertension

Lucie Miksová, Pavel Jansa

Centrum pro plicní hypertenzi, II. interní klinika kardiologie a angiologie, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice v Praze, Praha

Pulmonary arterial hypertension (PAH) is a rare, progressive, and life-threatening disease characterized by pathological remodeling of the pulmonary vasculature, ultimately leading to right ventricular failure. Despite substantial advances in diagnostic strategies and therapeutic options over recent decades, the prognosis of patients with PAH remains unfavorable, and a sustained low-risk profile cannot be achieved in a considerable proportion of patients even when treated according to current guideline recommendations. Current PAH-specific pharmacotherapy primarily targets the endothelin, prostacyclin, and nitric oxide-cGMP signaling pathways, with predominantly vasodilatory effects and only a limited impact on vascular remodeling. More recently, therapeutic options have expanded to include agents targeting additional pathophysiological mechanisms of the disease. This state-of-the-art review summarizes current concepts in PAH pharmacotherapy, with a focus on recent clinical trials and the resulting changes in the recommended treatment algorithm. Particular attention is paid to sotatercept, an activin signaling pathway inhibitor that restores the balance between proliferative and antiproliferative processes in the pulmonary vascular wall. Clinical studies have demonstrated its benefit when added to standard therapy, including improvements in functional and hemodynamic parameters as well as a reduction in the risk of major clinical events across all risk categories pretreated PAH patients. In addition, the results of the A DUE and AFFILIATE trials are discussed.

Keywords: activin signaling pathway inhibitor, specific pharmacotherapy, pulmonary arterial hypertension, sotatercept.

Accepted: March 25, 2026; Published: April 8, 2026  Show citation