Perioperative management of a patient with hereditary angioedema during off-pump coronary artery bypass graft surgery.

The case of a patient with hereditary angioedema (HAE), a rare, life-threatening disorder caused by reduced activity of the C1 esterase inhibitor, and requiring off-pump coronary artery bypass graft (OP-CABG) surgery, is presented. Perioperative management of patients with HAE who undergo complex cardiac surgical procedures are discussed, including an OP-CABG surgical approach to decrease complement activation, fresh-frozen plasma administration to increase C1 esterase inhibitor activity, and administration of reduced doses of heparin and protamine to minimize heparin-protamine complex formation.