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

Abstract

Pulmonary arterial hypertension (PAH) is a life-threatening condition characterised by the 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. These therapies regulate vascular tone through 3 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 anti-proliferative signalling of activin receptor type II (A/B), thus targeting a unique pathogenic pathway and promoting anti-proliferative effects on the pulmonary vasculature. Recently, it received approval from the European Medicines Agency for patients with PAH classified as World Health Organisation 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, including those at high risk. In anticipation of upcoming scientific guidelines and with the hope of improved outcomes for patients with PAH, an expert opinion for the treatment of Greek patients has been developed, focusing on the integration of this novel agent into the therapeutic algorithm.

Keywords: Activin signalling inhibitor; Expert opinion; Pulmonary arterial hypertension; Remodelling; Right ventricle; Sotatercept.