Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable condition caused by chronic thromboembolic involvement of the pulmonary vascular bed. Surgical endarterectomy is the preferred treatment, but not all patients are suitable for surgery. Medical therapy is used for patients who are inoperable or have residual PH after surgery. Anticoagulation is the mainstay of treatment for CTEPH, regardless of surgical candidacy. The use of endothelin receptor antagonists (ERAs), cyclic guanosine monophosphate (cGMP) phosphodiesterase 5 (PDE5) inhibitors, and prostanoids is considered for patients who cannot undergo surgery. The BENEFIT trial showed that bosentan improved pulmonary hemodynamics in patients with inoperable CTEPH. However, the trial did not show significant improvement in exercise capacity. Sildenafil also showed some benefit in improving PVR and exercise capacity in patients with inoperable CTEPH. Preoperative administration of prostanoids, ERAs, and/or PDE5 inhibitors should be coordinated with a center specializing in the surgical management of CTEPH. Surgical candidacy for PEA is determined by the presence of chronic organized thrombi involving the main, lobar, and/or proximal segmental pulmonary arteries, hemodynamic impairment proportional to the anatomically evident disease, and the absence of other significant comorbid conditions. PEA surgery involves a median sternotomy followed by the initiation of cardiopulmonary bypass. Postoperative complications include mediastinal hemorrhage, ventilator-associated pneumonia, wound infections, atrial arrhythmias, delirium, and phrenic nerve injury. Postendarterectomy reperfusion lung injury (RPLI) is a unique complication that may develop within the first 24 h of surgery and is associated with increased oxygen and ventilator requirements. Residual PH after PEA surgery is reported in 10–15% of patients. Operative mortality has been reduced to 4–7% at select centers of excellence. Patients with CTEPH should be referred to centers with considerable experience in the medical and surgical management of this disease. Coronary angiography is recommended before surgery in patients with risk factors for coronary atherosclerosis. Valve repair or replacement may be done during thromboendarterectomy. Tricuspid valve repair is rarely required and is performed only in patients with a severely damaged valve. Surgical closure of a patent foramen ovale may be performed if there is evidence of improvement in pulmonary hemodynamics. No potential conflicts of interest were reported. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License.Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable condition caused by chronic thromboembolic involvement of the pulmonary vascular bed. Surgical endarterectomy is the preferred treatment, but not all patients are suitable for surgery. Medical therapy is used for patients who are inoperable or have residual PH after surgery. Anticoagulation is the mainstay of treatment for CTEPH, regardless of surgical candidacy. The use of endothelin receptor antagonists (ERAs), cyclic guanosine monophosphate (cGMP) phosphodiesterase 5 (PDE5) inhibitors, and prostanoids is considered for patients who cannot undergo surgery. The BENEFIT trial showed that bosentan improved pulmonary hemodynamics in patients with inoperable CTEPH. However, the trial did not show significant improvement in exercise capacity. Sildenafil also showed some benefit in improving PVR and exercise capacity in patients with inoperable CTEPH. Preoperative administration of prostanoids, ERAs, and/or PDE5 inhibitors should be coordinated with a center specializing in the surgical management of CTEPH. Surgical candidacy for PEA is determined by the presence of chronic organized thrombi involving the main, lobar, and/or proximal segmental pulmonary arteries, hemodynamic impairment proportional to the anatomically evident disease, and the absence of other significant comorbid conditions. PEA surgery involves a median sternotomy followed by the initiation of cardiopulmonary bypass. Postoperative complications include mediastinal hemorrhage, ventilator-associated pneumonia, wound infections, atrial arrhythmias, delirium, and phrenic nerve injury. Postendarterectomy reperfusion lung injury (RPLI) is a unique complication that may develop within the first 24 h of surgery and is associated with increased oxygen and ventilator requirements. Residual PH after PEA surgery is reported in 10–15% of patients. Operative mortality has been reduced to 4–7% at select centers of excellence. Patients with CTEPH should be referred to centers with considerable experience in the medical and surgical management of this disease. Coronary angiography is recommended before surgery in patients with risk factors for coronary atherosclerosis. Valve repair or replacement may be done during thromboendarterectomy. Tricuspid valve repair is rarely required and is performed only in patients with a severely damaged valve. Surgical closure of a patent foramen ovale may be performed if there is evidence of improvement in pulmonary hemodynamics. No potential conflicts of interest were reported. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License.