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.