Chronic thromboembolic pulmonary hypertension is a progressive disease characterized by obstruction of the pulmonary vascular bed by insufficient resolution of thromboemboli leading to increased pulmonary vascular resistance with resultant right heart dysfunction and poor long-term survival. Estimates of cumulative incidence after an acute pulmonary embolism range from 1 to 4% within 2 years of an initial event. Current recommendations focus on screening patients being evaluated for pulmonary hypertension with ventilation/perfusion scans and then considering them for potentially curative surgery consisting of a pulmonary endarterectomy. Outcomes at high-volume surgical centers continue to improve and overall perioperative mortality is close to 2 to 4% with excellent reported long-term survival. Unfortunately, adherence with guidelines remains poor and patients with this potentially remediable disease are being missed. In addition, a significant number of patients who are diagnosed are not felt to be operative candidates based on location of disease, hemodynamics, or comorbidities. Several new advances may improve outcomes for such patients who are not operative candidates or who have persistent pulmonary hypertension after surgery, including medical therapy and balloon pulmonary angioplasty. Further studies are needed to answer questions about how best to incorporate these advances into our overall treatment algorithm.