Over the past decade, major advancements in the understanding of the
molecular mechanisms and pathophysiology of pulmonary hypertension
occurred in parallel with the discovery and development of new
therapies. Pharmacological agents that modulate the main
pathophysiological pathways of pulmonary arterial hypertension have
changed the course of this devastating disease by relieving symptoms and
improving and prolonging patients' lives.
The first part of the book covers definition, classification,
pathophysiology, pathology, biomarkers and animal models of pulmonary
hypertension, thus laying the conceptual basis for what follows. The
middle section provides an overview of the established therapies, such
as calcium channel blockers, prostanoids, endothelin receptor
antagonists, phosphodiesterase-5 inhibitors and inhaled nitric oxide.
The concluding section explores novel pathways and emerging therapeutic
approaches including soluble guanylate cyclase stimulators, Rho-kinase
inhibitors, inhibitors of serotonin receptors and transporters, peptide
growth factors, vasoactive peptides, modulators of redox equilibrium and
cyclic nucleotides homeostasis, as well as immunosuppressive and
anti-proliferative agents. Particular attention is given to clinical
applications of these experimental therapies.