LAUSR

BACKGROUND Patients with a Fontan circulation are at risk of renal dysfunction. We analyzed cross-sectional data in pediatric and adult Fontan patients in order to assess the accuracy of commonly used serum creatinine-based methods in estimating glomerular filtration rate (GFR). METHODS A total of 124 Fontan patients (58 children, 66 adults) were enrolled across three study centers. Measurement of GFR (mGFR) using in vivo 99m Tc-DTPA clearance was performed. Various serum creatinine-based equations were used to calculate estimated GFR (eGFR). RESULTS Mean mGFR was 108 ± 28 mL/min/1.73 m2 in children and 92 ± 20 mL/min/1.73 m2 in adults. Fourteen children (25%) and 28 adults (45%) had an mGFR <90 mL/min/1.73 m2 . There was no significant correlation between mGFR and eGFR (Schwartz) in children (r = 0.22, P = .1), which substantially overestimated mGFR (bias 50.8, 95%CI: 41.1-60.5 mL/min/1.73 m2 , P < .0001). The Bedside Schwartz equation also performed poorly in the children (r = 0.08, P = .5; bias 5.9, 95%CI: -2.9-14.6 mL/min/1.73 m2 , P < .0001). There was a strong correlation between mGFR and both eGFR (CKD-EPI) and eGFR (MDRD) in adults (r = 0.67, P < .0001 in both cases), however, both methods overestimated mGFR (eGFR(CKD-EPI):bias 23.8, 95%CI: 20-27.6 mL/min/1.73 m2 , P < .0001; eGFR (MDRD):bias 16.1, 95%CI: 11.8-20.4 mL/min/1.73 m2 , P < .0001). None of the children with an mGFR <90 mL/min/1.73 m2 had an eGFR (Schwartz) <90 mL/min/1.73 m2 . Sensitivity and specificity of eGFR (CKD-EPI) and eGFR (MDRD) for mGFR <90 mL/min/1.73 m2 in adults were 25% and 92% and 39% and 100%, respectively. CONCLUSIONS This study identifies the unreliability of using creatinine-based equations to estimate GFR in children with a Fontan circulation. The accuracy of formulas incorporating cystatin C should be further investigated and may aid noninvasive surveillance of renal function in this population.