Pulmonary thromboembolectomy in congenital antithrombin III deficiency associated with acute pulmonary embolism -report of a case-.

Antithrombin III (AT III) deficiency is a rare hereditary disease that predisposes to thromboembolic complications. We report a case of AT III deficiency complicated with acute pulmonary thromboembolism, successfully treated with emergency pulmonary thromboembolectomy after insertion of an inferior vena cava filter. AT III activity before treatment was found to be 44% of normal value and remained less than 50% of normal throughout the postoperative course. In his family line, both the patient's aunt and deceased father had a history of pulmonary infarction. AT III activity of the patient's aunt was 47 to 58% of normal value. The patient was discharged on the 15th day after surgery and has been doing well for four years receiving warfarin as anticoagulant therapy. Careful follow-up is essential for early detection of the recurrent pulmonary thromboembolism resulting in pulmonary hypertension and/or right heart failure.