Cor Vasa 2025, 67(2):295-300 | DOI: 10.33678/cor.2025.046

(Current treatment options of pulmonary arterial hypertension in the Czech Republic, its limitations and perspectives, update of the therapeutic algorithm for pulmonary arterial hypertension. Expert consensus statement of the Working Group on Pulmonary Circulation of the Czech Society of Cardiology)

Pavel Jansa^a, David Ambrož^a, Marián Felšőci^b, Lucie Miksová^a, Jan Přeček^c, Hikmet Al-Hiti^d, Radek Pudil^e, Martin Hutyra^c

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

^b Interní kardiologická klinika, Lékařská fakulta Masarykovy univerzity a Fakultní nemocnice Brno, Brno, Česká republika

^c I. interní klinika - kardiologická, Lékařská fakulta Palackého univerzity v Olomouci a Fakultní nemocnice Olomouc, Olomouc, Česká republika

^d Klinika kardiologie, Institut klinické a experimentální medicíny, Praha, Česká republika

^e I. interní kardioangiologická klinika, Lékařská fakulta v Hradci Králové Univerzity Karlovy a Fakultní nemocnice Hradec Králové, Hradec Králové, Česká republika

Pulmonary hypertension is defined as a syndrome characterized by an increase of the mean pulmonary artery pressure >20 mmHg. Pulmonary arterial hypertension (PAH) is a rare disease that accounts for approximately 1% of all cases of chronic pulmonary hypertension. In the pathophysiology, the key factor is initially vasoconstriction, followed by remodeling of pulmonary arterioles, accompanied by an increase of pulmonary vascular resistance and the right ventricle chronic pressure overload. In the treatment of PAH, medical therapy is dominant. Patients with a positive vasoreactivity testing are indicated for treatment with high doses of calcium channel blockers. In the case of a negative test (in most patients), medical therapy with prostanoids, prostacyclin receptor agonists, endothelin receptor antagonists, phosphodiesterase 5 inhibitors, and soluble guanylate cyclase stimulators is indicated (initial combination therapy for patients without cardiopulmonary comorbidities, initial monotherapy for patients with cardiopulmonary comorbidities, repeated reevaluation during follow-up and appropriate modification of treatment). The still high mortality of patients currently treated for PAH according to these recommendations documents the urgent need not only for earlier diagnosis but also for a more effective treatment. A major change in the medical therapy of PAH since the last 2022 ESC/ERS guidelines is sotatercept, which leads to the induction of balanced signaling between the proliferative effects of the activin signaling pathway and the antiproliferative effects of bone morphogenetic protein. By binding to activin receptor ligands, sotatercept modulates proliferation and thus represents the first therapeutic option to predominantly influence vascular remodeling of pulmonary arterioles, which is a key pathophysiological mechanism in PAH.

Keywords: Activin, Bone morphogenetic protein, Medical therapy, Pulmonary arterial hypertension, Sotatercept

Received: March 17, 2025; Revised: March 17, 2025; Accepted: March 18, 2025; Prepublished online: June 2, 2012; Published: May 1, 2025  Show citation