Over the past 4 decades, chronic thromboembolic pulmonary hypertension has evolved from an autopsy curiosity to a potentially correctable form of pulmonary hypertension. Approximately 2500 thromboendarterectomy procedures have now been performed worldwide with mortality rates reported by established programs experienced in the management of this disease process falling to a range of 4 to 8%. Following a successful pulmonary thromboendarterectomy, substantial improvement, and at times normalization, can be achieved in right ventricular function, gas exchange, exercise capacity, and quality of life. Reperfusion lung injury and residual postoperative pulmonary hypertension remain the major causes of mortality in patients undergoing the procedure.