HYPERURICEMIA IN AN INFANT WITH TAUSSIG-BING ANOMALY AND INTERRUPTIONOF THE AORTIC-ARCH

Hyperuricemia is commonly recognized in adolescents and adults with cy anotic congenital heart disease. We report a case of a male infant wit h hyperuricemia, Taussig-Bing anomaly, and interruption of the aortic arch. The patient underwent correction of interrupted aortic arch and pulmonary arterial banding at the age of 7 days. Hyperuricemia appeare d when he was 2 months old (max 17.7 mg/dl) and persisted until he und erwent a Jatene operation at the age of 10 months. The hyperuricemia i mproved gradually after the disappearance of hypoxia and polycythemia. The laboratory findings suggest that hyperuricemia can result from ur ic acid overproduction due to secondary polycythemia, impairment of ur ic acid excretion by the kidney, or the acceleration of anaerobic meta bolism. Allopurinol and benzbromarone together were partially effectiv e treatments for hyperuricemia in this patient with cyanotic congenita l heart disease.