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Chronic thromboembolic pulmonary hypertension is a fatal disease if left untreated, and pulmonary endarterectomy (PEA) is the potentially curable treatment of choice. We aimed to estimate the current in-hospital mortality rate, complication rate and long-term survival for patients with chronic thromboembolic pulmonary hypertension undergoing PEA in Denmark.All chronic thromboembolic pulmonary hypertension patients who underwent PEA in the period 1994 till 2016 were consecutively enrolled in our single-centre study. All patients were followed from PEA until death or end of study. Kaplan-Meier survival analysis was used to estimate the 3-, 5- and 10-year survival rates with 95% confidence interval (CI).In total, 239 patients were operated in the study period. A significant reduction in mean pulmonary arterial pressure from 48 mmHg to 33 mmHg, and pulmonary vascular resistance from 800 dynes s cm−5 to 289 dynes s cm−5, was observed during the first postoperative day after PEA. Overall, in-hospital mortality rate was 8.4%. A significantly lower mortality rate in the late decade (2005-2016) compared with the early decade (1994-2004) was observed (4.3% vs 22.6%, P < 0.001). In-hospital mortality during the last 5 years (n = 80) was 2.5%. Three-, 5- and 10-year survival rates were 84% (CI: 77.8-88), 77% (CI: 70.7-82.7) and 62% (CI: 53-69.1), respectively. The majority of patients improved in World Health Organization functional class from III/IV to I/II and significantly increased their 6-min walking distance.PEA in Denmark is associated with a low in-hospital mortality rate and significant improvements in both haemodynamics and exercise capacity. Long-term survival is excellent and similar to high-volume international centres.