This study examines the exacerbating factors of hyeruricaemia in patie
nts with cyanotic congenital heart disease (CCHD). We studied 59 CCHD
patients aged 1 month-30 years. The following variables were assessed:
serum uric acid levels, red blood cell count, haemoglobin, hematocrit
, partial oxygen pressure and arterial oxygen saturation. Uric acid ex
cretion and renal function were also measured in ten patients with ser
um levels of uric acid greater than 8 mg/dl (hyperuricaemia group). Se
rum uric acid level correlated significantly with age and severity of
polycythaemia. However, it did not correlate with partial oxygen press
ure or arterial oxygen saturation. Uric acid excretion was measured in
hyperuricaemia group. Urinary uric acid excretion (24 h) was within n
ormal limits in infants but markedly lower in patients over 15 years o
f age. The aetiology of hyperuricaemia and decreased uric acid fractio
nal excretion and clearance in infants appears to be secondary to dimi
nished excretion of uric acid in concert with uric acid overproduction
. Hyperuricaemia in adolescents and adults with CCHD, however, results
mainly from age-related impairment of uric acid excretion.