The last 4 decades have witnessed dramatic changes in the treatment of patients with pulmonary hypertension. And with the advances in cardiothoracic surgical techniques, selected patients with chronic thromboembolic pulmonary hypertension (CTEPH) are afforded a surgical remedy for their disease. In the vast majority of these patients, surgical endarterectomy of chronic thromboemboli from the pulmonary vascular bed effectively treats even severe pulmonary hypertension, and as a result, the debilitating symptoms of right heart dysfunction. This article reviews the natural history of chronic thromboembolic disease and outline a suggested diagnostic approach to determine whether a patient with chronic thromboembolic disease might be an appropriate surgical candidate. Pulmonary thromboendarterectomy surgery, and the growing information on the use of pharmacotherapy in inoperable CTEPH, are also reviewed.