Revisiting treatment of pulmonary arterial hypertension in the current era: A Greek scientific document.

Abstract

Pulmonary arterial hypertension (PAH) is a life-threatening condition characterized by excessive proliferation of pulmonary artery vessels. Despite significant advancements in treatment strategies over recent years, mortality rates remain high. The current treatment strategy focuses on risk assessment both at the time of diagnosis and during follow-up. It involves the initial use of combination therapies targeting PAH, which regulate vascular tone through three main pathways: the endothelin pathway, the nitric oxide/cyclic guanosine monophosphate pathway, and the prostacyclin pathway. Sotatercept, a fusion protein that binds to ligands of the transforming growth factor-β superfamily, rebalances the pro- and antiproliferative signalling of activin receptor type II A/B, thus targeting a unique pathogenic pathway and promoting antiproliferative effects on the pulmonary vasculature. Recently, it received approval from the European Medicines Agency for PAH patients classified as WHO functional class II or III. Proceedings from the latest World Symposium on Pulmonary Hypertension stress the importance of adding sotatercept to the treatment regimen for the majority of patients during follow-up. In anticipation of upcoming scientific guidelines and with the hope of improved outcomes for PAH patients, an expert opinion for the treatment of Greek patients has been developed, focusing on the integration of this novel agent into the therapeutic algorithm.