Pulmonary hypertension and cor pulmonale

An ever-expanding body of information has provided new insights into the pathophysiologic mechanisms contributing to pulmonary hypertensive disease. The pulmonary endothelial cell has been shown to have a central role in both the maintenance of normal vascular tone and in the pathogenesis of small vessel changes. The relationship between endothelium-derived mediators such as nitric oxide, prostacyclin, and endothelin-1 is likely to be important in the development of abnormal vasomotor tone and structure in the pulmonary vascular bed. Stimulated by this evolving understanding of pulmonary vascular physiology, recent literature abounds with references to novel therapeutic strategies in the care of patients who have both primary and secondary forms of pulmonary hypertension. Advances in surgical technologies have also expanded the therapeutic options for selected groups of pulmonary hypertensive patients. This article highlights recent developments in the understanding of pulmonary vascular pathophysiology and examines strategies for evaluation and treatment of pulmonary hypertension and cor pulmonale.