ABSTRACT
Pulmonary hypertension (PH) is defined as a mean pulmonary arterial pressure of
greater than 25 mmHg at rest or greater than 30 mmHg with exercise with a pulmonary
vascular resistance greater than 3.0 mmHg/l/min. Recently, the World Health
Organization convened a conference to re-classify PH based on underlying mechanisms
of pathophysiology and pathobiology. Thus, the old terminology of primary and
secondary PH has been replaced; the current classification subdivides PH into subgroups
of pulmonary arterial hypertension, which includes idiopathic pulmonary arterial
hypertension previously known as primary PH, or PH associated with connective tissue
disease and chronic systemic-to-pulmonary shunt, left heart disease, pulmonary vascular
disorders, hypoxemia or respiratory disorders, chronic thromboembolic disease, and
other miscellaneous disorders. The focus of this chapter is PH in the setting of
advanced heart failure, both from chronic systemic-to-pulmonary shunt and left heart
disease.