Pulmonary endarterectomy: the lancet first, tears for pills

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease due to the incomplete resolution of pulmonary emboli, leading to right heart failure, with a poor survival. Pulmonary endarterectomy (PEA) is the operation of choice for CTEPH. As there are no well-defined criteria to discriminate surgically accessible from inaccessible obstructive lesions, the operability assessment relies on the surgeon's experience. The recommended algorithms to perform a correct diagnosis of CTEPH still suggest the lung ventilation/perfusion scan, despite advances in computed tomography with 3-D reconstruction and magnetic resonance imaging. Selective pulmonary angiography is the gold standard to assess operability in CTEPH. Medical therapy should not be considered an alternative to PEA, as it should be reserved to patients with either peripheral disease, deemed inoperable by an experienced PEA surgeon, or persistent/recurrent pulmonary hypertension after PEA. Lung transplantation, when indicated, still represents a viable option for patients with either inoperable CTEPH or CTEPH with concomitant severe parenchymal lung disease that contraindicates PEA. The outcome of operable CTEPH is still best predicted after surgery. Remarkably, the recovery of exercise capacity is not as immediate as hemodynamic improvement, underlining the importance of early identification of surgical candidates before physical deconditioning.