Pulmonary vascular disease and transplant in advanced lung diseases

Increased pulmonary artery pressures commonly occurs in advanced lung diseases and is the second most common cause of pulmonary hypertension. Pulmonary hypertension due to advanced lung diseases include chronic obstructive lung disease, restrictive lung disease, disease with mixed restrictive/obstructive pattern, hypoxemia without lung disease, and developmental lung disorders. Evaluation for pulmonary hypertension in patients with advanced lung disease should be pursued when there is high suspicion. Confirmation of pulmonary hypertension diagnosis given by right heart catheterization is likely to impact management, including a trial of pulmonary vasodilators and referral for lung transplantation.