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Pulmonary hypertension is one of the lethal complications in the antiphospholipid syndrome due to chronic recurrence of embolism or thrombosis. We experienced a 19-year-old man suffering from severe chronic thromboembolic pulmonary hypertension (CTEPH) caused by primary antiphospholipid syndrome. According to the guideline, pulmonary thromboendarterectomy was decided on after combined therapy with warfarin, beraprost and oxygen inhalation had not been effective enough to improve severe CTEPH. By an operation, the mean pressure of the pulmonary artery was decreased from 50 mmHg to 16 mmHg, while his New York Heart Association (NYHA) functional class classification significantly improved from class III to class I. We concluded that pulmonary thromboendarterectomy could dramatically improve hemodynamic indices, NYHA functional status and quality of life in patients with CTEPH.

Successful Thromboendarterectomy for Severe Chronic Thromboembolic Pulmonary Hypertension Caused by Primary Antiphospholipid Syndrome