ObjectiveTo summarize the clinical characteristics and the long-term results of pulmonary thromboendarterectomy (PTE) in the chronic thromboembolic pulmonary hypertension (CTEPH) patients with unilateral main pulmonary artery occlusion.MethodsWe retrospectively analyzed the clinical data of 15 CTEPH patients with unilateral main pulmonary artery occlusion in Fuwai Hospital between 2004 and 2018. There were 11 males and 4 females aged 34.1±12.0 years at operation.ResultsThe mean circulatory arrest was 31.1±12.1 minutes. The ICU stay was 5 (2-29) d. The hospital stay was 15 (8-29) d. There was no hospital death. There was a decline in systolic pulmonary artery pressures (sPAP, 69.9±27.9 mm Hg to 35.1±9.7 mm Hg, P=0.020) after surgery. On postoperative V/Q scan, only 6 patients (40.0%) had significant improvement in reperfusion (≥75% estimated) of the occluded lung. There was no death during the median observation period of 49 months follow-up, while 2 patients had recurrence of pulmonary embolism.ConclusionCTEPH patients with unilateral main pulmonary artery occlusion represent a challenging cohort. PTE is a curative resolution in both early- and long- term results, although there is a high requirement of perioperative management and a high risk of postoperative complications and rethrombosis.