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Focal segmental glomerulosclerosis in patients after Fontan operation: is this a Fontan-associated renal disease?

Takashi Furuta1, Jun Muneuchi1, Yuichiro Sugitani1

  • 1Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan.

Cardiology in the Young
|September 15, 2021
PubMed

Insights

Adults undergoing the Fontan operation face risks of kidney disease. Focal segmental glomerulosclerosis may develop due to the operation's circulatory effects, termed Fontan-associated renal disease.

Area of Science:

  • Cardiology
  • Nephrology
  • Pediatric Cardiology

Background:

  • The Fontan operation is a palliative surgical procedure for complex congenital heart defects.
  • While Fontan survivors experience improved survival rates, long-term organ-specific complications remain a concern.

Observation:

  • Two adult cases of focal segmental glomerulosclerosis (FSGS) following Fontan operation are presented.
  • These patients developed FSGS despite acceptable Fontan circulation survival.

Findings:

  • Focal segmental glomerulosclerosis in these cases is hypothesized to stem from Fontan-associated hemodynamic issues.
  • Contributing factors include chronic venous congestion, systemic hypoxia, and hyperviscosity.
  • This condition is proposed to be termed Fontan-associated renal disease (FARD).

Implications:

  • This study highlights a potential renal complication in adult Fontan survivors.
  • Understanding Fontan-associated renal disease is crucial for long-term management and monitoring of these patients.
  • Further research is needed to confirm the pathophysiology and establish treatment guidelines for FARD.

Keywords:
CHDFontan operationfocal segmental glomerulosclerosismesangial proliferative glomerulonephritis

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