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Hyperuricemia in an infant with Taussig-Bing anomaly and interruption of the aortic arch
Y Hayabuchi1, S Matsuoka, Y Takahashi
1Department of Pediatrics, University of Tokushima School of Medicine, Japan.
Insights
Hyperuricemia in infants with cyanotic congenital heart disease can stem from polycythemia or impaired kidney function. Treatment with allopurinol and benzbromarone showed partial effectiveness in this case.
Area of Science:
- Pediatrics
- Cardiology
- Nephrology
Background:
- Cyanotic congenital heart disease (CCHD) is often associated with hyperuricemia in older patients.
- The Taussig-Bing anomaly and interruption of the aortic arch are complex congenital heart defects.
Observation:
- A male infant with Taussig-Bing anomaly and interrupted aortic arch developed significant hyperuricemia (max 17.7 mg/dl) at 2 months of age.
- Hyperuricemia persisted until a Jatene operation at 10 months, improving post-operatively with resolution of hypoxia and polycythemia.
Findings:
- The infant's hyperuricemia was likely multifactorial, potentially involving uric acid overproduction from secondary polycythemia, reduced renal excretion, and accelerated anaerobic metabolism.
- Combined treatment with allopurinol and benzbromarone provided partial efficacy in managing the hyperuricemia.
Implications:
- This case highlights that hyperuricemia can occur in infants with complex cyanotic congenital heart disease.
- Understanding the pathophysiology is crucial for managing hyperuricemia in these vulnerable patients.
- Further research may explore optimal therapeutic strategies for hyperuricemia in pediatric CCHD.
Abstract:
Hyperuricemia is commonly recognized in adolescents and adults with cyanotic congenital heart disease. We report a case of a male infant with 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 appeared when he was 2 months old (max 17.7 mg/dl) and persisted until he underwent a Jatene operation at the age of 10 months. The hyperuricemia improved gradually after the disappearance of hypoxia and polycythemia. The laboratory findings suggest that hyperuricemia can result from uric acid overproduction due to secondary polycythemia, impairment of uric acid excretion by the kidney, or the acceleration of anaerobic metabolism. Allopurinol and benzbromarone together were partially effective treatments for hyperuricemia in this patient with cyanotic congenital heart disease.