ABSTRACT
Our understanding of the pathophysiology of PAH is still in its infancy, although significant advances have been made based on molecular science, genetics and the understanding of the clinico-pathological interactions recognized in the WHO classification scheme. Undoubtedly, the pathophysiology of PAH is complex, pivoting around the concepts of vasoconstriction, vascular remodelling and thrombosis. Vasoactive substances, growth factors, inflammatory mediators and components of the clotting/coagulation system are all involved to varying degrees.20 This complex interplay is only just being decoded, but already there have been both real and potential therapeutic targets unearthed.
