Surgical treatment of atrial septal defect in a patient with familial antithrombin-III deficiency.

PURPOSE: A 41-year-old woman with familial antithrombin-III deficiency was admitted to our hospital for patch closure of an atrial septal defect. Antithrombin-III activity was 43% and its antigen level was 12.2 mg/dl, was diagnosed so type I antithrombin-III deficiency was diagnosed.

METHODS

A dose of 2500 U of antithrombin-III concentrate was administered at 1 day before surgery, 1 hour before surgery, and 1 day after surgery. Heparinazation was performed at 200 IU/kg and the activated clotting time increased from a baseline of 140s to 622s. After establishing cardiopulmonary bypass and cardioplegic arrest, closure of the septal defect was done with a pericardial patch.

RESULTS

The activated clotting time was maintained at more than 400s during cardiopulmonary bypass. There were no intraoperative complications and the postoperative course was uneventful.

CONCLUSION

In patients with familial antithrombin-III deficiency, administration of antithrombin-III concentrate is effective when cardiopulmonary bypass is required.