ABSTRACT

Pathologically elevated pulmonary artery pressure may occur in the setting of elevated pulmonary vascular resistance, excessive pulmonary blood flow and/or pulmonary venous hypertension. Each of these disease phenotypes may be present both in the acute and chronic state. The therapeutic approach to manage elevated pulmonary artery pressure is dependent on the etiology; therefore, delineation of the biological phenotype is essential in the provision of optimal care. A non-judicious approach towards use of pulmonary vasodilators, based on the assumption that all pulmonary hypertension is exclusively related to elevated pulmonary vascular resistance, may be detrimental in the setting of flow or pulmonary venous disease. In this chapter we illustrate the distinguishing features of each phenotype and propose an approach to diagnosis and therapy, guided primarily by echocardiography and in some cases cardiac catheterization.