EXPRESS: Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: state-of-the-art 2020

Pulmonary endarterectomy (PEA) is the treatment of choice for patients with operable CTEPH as it is potentially curative. In expert centers that conduct > 50 PEA procedures per year, peri- and post-surgical mortality rates are very low and long-term outcomes are excellent, with 3-year post-operative survival of > 80%. Therapeutic decisions in CTEPH are based largely on the location of the arterial obstruction, with PEA for obstructions in main, lobar, and segmental vessels, and balloon pulmonary angioplasty (BPA) and medical therapy for small-vessel disease. Medical therapy is also an option for patients with persistent/recurrent pulmonary hypertension after PEA or BPA. With increasing surgical experience and improvements in instruments and procedures, an increasing number of patients are now considered operable who would previously have been inoperable, including some patients with subsegmental disease. At our University, around 200 PEA procedures are performed every year and several advances have been developed, including resection of more distal disease, availability of PEA to patients previously considered to be at too high risk for surgery, improved management of post-PEA complications, and minimally invasive PEA. PEA can be combined with other treatment modalities, including BPA, medical therapy for persistent/recurrent pulmonary hypertension after PEA, and medical therapy or BPA as bridging therapy before surgery. Data on these combinations are, however, limited. Combination treatment should therefore be considered on an individual patient basis. In the future, however, multimodal therapy with PEA, BPA, and/or medical therapy is likely to be an important treatment option for many patients.