Pulmonary hypertension related to respiratory disease

Cor pulmonale is a condition characterized by right ventricular hypertrophy, dilation, or both, occurring as a result of parenchymal lung disease, impaired respiratory neuromuscular mechanics, or altered ventilatory drive. Changes in right ventricular structure and function are a consequence of elevations in pulmonary arterial pressure and pulmonary vascular resistance, ie, pulmonary hypertension. Although alveolar hypoxia leading to pulmonary vasoconstriction may play an important role in the pathogenesis of pulmonary hypertension in respiratory disease, other mechanisms are likely to be involved. Recent studies have estimated that the prevalence of pulmonary hypertension complicating various forms of respiratory disease may be higher than previously thought. Although the magnitude of pulmonary arterial pressure elevation is often modest, the impact of pulmonary hypertension upon survival is significant. The role of pharmacologic agents approved for pulmonary arterial hypertension is yet to be defined. In this article, we (1) review the epidemiology and pathophysiology of pulmonary hypertension related to respiratory diseases, (2) outline recommendations for diagnostic evaluation, and (3) provide recommendations for therapy.