Assessment of Kidney Function in Survivors Following Fontan Palliation

Sheena Sharma1, Rebecca L Ruebner1, Susan L Furth1

  • 1Division of Nephrology and, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Insights

Long-term survivors of the Fontan operation show early signs of kidney disease, with 10% having reduced kidney function and elevated parathyroid hormone levels. These findings suggest potential chronic kidney disease (CKD) in this patient population.

Area of Science:

  • Pediatric Cardiology
  • Nephrology
  • Congenital Heart Disease Research

Background:

  • The Fontan operation is a critical palliative surgery for single ventricle congenital heart disease.
  • Long-term renal outcomes following the Fontan procedure are not well-established.
  • Understanding kidney function in survivors is crucial for comprehensive long-term care.

Purpose of the Study:

  • To evaluate long-term kidney function in individuals who have undergone the Fontan operation.
  • To investigate the prevalence of chronic kidney disease (CKD) in this cohort.
  • To compare renal function markers between Fontan survivors and healthy controls.

Main Methods:

  • A retrospective cohort study compared 68 Fontan survivors with 70 healthy children.
  • Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate (eGFR) <90 mL/min/1.73 m².
  • Creatinine and cystatin C-based equations were used to calculate eGFR; proteinuria and parathyroid hormone levels were also assessed.

Main Results:

  • While median eGFR levels were comparable between groups, 10% of Fontan survivors exhibited an eGFR <90 mL/min/1.73 m².
  • Fontan survivors had significantly higher median intact parathyroid hormone levels compared to controls (59.4 vs. 23.4 pg/mL).
  • Proteinuria was observed in 10% of the Fontan group, compared to 4.7% in controls, although this difference was not statistically significant.

Conclusions:

  • A notable proportion of long-term Fontan survivors demonstrate indicators of early kidney disease, including reduced eGFR and elevated parathyroid hormone.
  • These findings suggest a potential for developing chronic kidney disease (CKD) in the post-Fontan population.
  • Further longitudinal studies are warranted to monitor kidney function and identify risk factors for CKD in this unique cohort.
Abstract

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