Pulmonary arterial hypertension: pathobiology, diagnosis, treatment, and emerging therapies.

Pulmonary hypertension is a rare disorder caused by vasoconstriction of the pulmonary arteries that leads to elevation of pulmonary vascular resistance, right ventricular failure, and ultimately death. Hypertrophy and proliferation of the pulmonary vascular endothelium leads to remodeling of this vascular system, resulting in a progressive disorder. In the past decade the molecular mechanisms and pathobiology of this disorder has become clearer. In addition, a host of new medical treatments and therapies are now available for what has been previously known to be a devastating disorder. Although much needs to be learned, this review will discuss our current knowledge, results of clinical trials, along with treatment options and emerging therapies available for the treatment of this disorder.