Chronic, major-vessel thromboembolic disease represents a potentially correctable form of pulmonary hypertension. A thorough understanding of the unique problems associated with each aspect of care in these patients is essential to ensure a minimal perioperative mortality and satisfactory long-term outcome. This article reviews the cause, natural history, evaluation, surgical management, and postoperative course of patients with chronic thromboembolic pulmonary hypertension undergoing thromboendarterectomy.