Pulmonary Hypertension

Clinical Classification of Pulmonary Hypertension

Pulmonary arterial hypertension (classified as Group 1) may occur in several forms. Pulmonary arterial hypertension that exists by itself and has no known underlying cause is called idiopathic pulmonary arterial hypertension (IPAH). Many patients have pulmonary arterial hypertension in association with an underlying disease such as scleroderma or congenital heart disease (called Associated PAH, APAH). At Mayo Clinic, treatment for pulmonary arterial hypertension includes medications (Food and Drug Administration approved or investigational), and, in rare instances, lung transplant.

Pulmonary hypertension may also occur with left-sided heart disease (pulmonary venous hypertension, classified as Group 2). Other underlying causes for pulmonary hypertension (classified as Group 3) are lung diseases such as fibrosis and emphysema, or sleep disorders. The condition may also occur with blood clots in the lung (chronic thromboembolic pulmonary hypertension, classified as Group 4) or multifactorial disorders (classified as Group 5). In some instances, successful treatment of the underlying condition may improve or cure the pulmonary hypertension.

Read more about the most recent classification system of pulmonary hypertension on PubMed.