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1.
Emerging therapies and new directions in the treatment of pulmonary arterial hypertension
Grzegorz Kopec, Andris Skride, Egle Ereminiene, Iveta Šimkova, Roxana Enache, Miroslav Samaržija, Barbara Salobir, Pavel Jansa, 2025, pregledni znanstveni članek

Povzetek: Pulmonary arterial hypertension (PAH) is a severe and progressive disease with limited survival prospects under currently available therapies. Since the 2022 edition of the European Society of Cardiology and European Respiratory Society guidelines on pulmonary hypertension, substantial clinical evidence has emerged, supporting a new treatment algorithm for PAH as presented at the 7th World Symposium on Pulmonary Hypertension 2024 and the following proceeding papers. Key updates include the introduction of sotatercept as a second-line therapy leading to a revised definition of maximal medical therapy now encompassing agents from four therapeutic groups (phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin pathway agents, and sotatercept), instead of three (phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin pathway agents). Other novelties include the elimination of a distinct pathway for patients with cardiopulmonary comorbidities in favor of an individualized approach, a reduction in the initial patient assessment risk categories from three to two, and a follow-up interval shortened from 3–6 months to 3–4 months post-treatment initiation. This review presents these advancements and emphasizes the need for their widespread implementation in clinical practice. At the end, we present new opportunities and challenges in the treatment of pulmonary arterial hypertension in eight Central and Eastern European countries.
Ključne besede: activin signaling inhibitors, novel therapies, risk assessment, treatment strategy, sotatercept
Objavljeno v DiRROS: 15.12.2025; Ogledov: 402; Prenosov: 651
.pdf Celotno besedilo (175,71 KB)
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2.
Long-term treatment with subcutaneous treprostinil in patients with severe inoperable chronic thromboembolic pulmonary hypertension in the multimodal therapy era (data from CTREPH study open label extension)
Pavel Jansa, Roela Sadushi-Kolici, Nika Skoro-Sajer, Grzegorz Kopec, Iveta Šimkova, Regina Steringer-Mascherbauer, Barbara Salobir, 2025, izvirni znanstveni članek

Povzetek: The aim of the open label extension (OLE) of CTREPH study was to characterize multimodal treatment in patients with severe inoperable CTEPH, to describe long-term subcutaneous (SC) treprostinil safety and tolerability, and to evaluate change in functional class and exercise capacity over 24 months since completion of the blinded phase of CTREPH. The target population in the OLE consisted of patients who completed 24 weeks of blinded treatment with either high-dose treprostinil of around 30 ng/kg/min (former high-dose group), or low-dose treprostinil of around 3 ng/kg/min (former low-dose group) in the CTREPH study. From the start of OLE, treprostinil dose and any additional therapy were chosen according to the standard of care and physician's discretion. Out of 47 enrolled patients, 20 patients received other PH drugs during OLE and 17 patients underwent at least 1 BPA session. Number of treprostinil-related AEs was substantially higher in the former low-dose group in comparison to the former high-dose group. Related AEs were also more frequent during the first 6 months of the preceding blinded trial than over 24 months of OLE, especially infusion site pain and all local infusion site reactions. No new safety signal was detected. Evaluated clinical outcomes show sustained benefit from long-term treprostinil treatment. Long-term SC treprostinil is a safe and effective component of multimodal treatment for patients with severe CTEPH. Patients who tolerate treprostinil after initiation are likely to continue tolerating it over time, with the clinical benefit maintained over 24 months.
Ključne besede: chronic thromboembolic pulmonary hypertension, multimodal treatment, subcutaneous treprostinil
Objavljeno v DiRROS: 15.12.2025; Ogledov: 1034; Prenosov: 242
.pdf Celotno besedilo (693,79 KB)
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