Laura C. Price, Colm McCabe, Jason Weatherald

European Respiratory Journal 2023 62: 2301513; DOI: 10.1183/13993003.01513-2023

Extract

Pulmonary arterial hypertension (PAH) is a severe progressive disease characterised by obliterative vascular remodelling and increased resistance in small and medium-sized pulmonary arteries (PAs). Contributing factors to PAH pathogenesis include genetic mutations such as those in bone morphogenetic protein receptor type 2 (BMPR2), perivascular inflammation, systemic immune dysregulation, and an imbalance of pulmonary vascular cell proliferation versus apoptosis [1]. PAH remains a malignant condition, despite therapeutic advances, with usually irreversible remodelling of resistance vessels. Morbidity relates to exercise-related breathlessness symptoms, reduced quality of life and, ultimately, increased mortality follows the onset of right ventricular (RV) dysfunction due to uncoupling of the RV–PA unit [2].