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1 Medicine, SUNY, Stony Brook, Stony Brook, New York, United States; Research, Northport VAMC, Northport, New York, United States
* To whom correspondence should be addressed. E-mail: sami.i.said{at}stonybrook.edu.
Pulmonary Arterial Hypertension (PAH), presenting as the only identifiable disease and known as either Familial or Idiopathic (Primary) Pulmonary Hypertension, is a relatively rare and highly fatal condition. Major advances have recently been made in our understanding of the mechanisms underlying this disorder. Genetic mutations of the TGF-beta / Bone Morphogenetic Protein Receptor II pathways predispose to the disease, but are often insufficient to cause the clinical condition. This review summarizes current knowledge relating to the role of vasoactive compounds and certain growth factors, with special reference to their influence on vascular tone, response to hypoxia, vascular remodeling, and other processes involved in the pathogenesis of PAH; 2) briefly discusses experimental models of the disease; and 3) highlights the pharmacologic basis for several recently introduced therapeutic agents.
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