Development of nephropathy in an adult patient after Fontan palliation for cyanotic congenital heart disease

Kaori Hayashi, Akinori Hashiguchi, Masako Ikemiyagi, Hirobumi Tokuyama, Shu Wakino, Hiroshi Itoh

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)


Cyanotic congenital heart disease is occasionally associated with kidney dysfunction, which is known as cyanotic nephropathy or cyanotic glomerulopathy. The clinical presentation of cyanotic nephropathy includes proteinuria, decreased estimated glomerular filtration rate, hyperuricemia, thrombocytopenia, or polycythemia. Although advances in surgical procedures have improved the prognosis of cyanotic congenital heart diseases, adult cases of cyanotic nephropathy are still rare, and there are few reports of kidney biopsy in adults with cyanotic nephropathy. Here, we present the case of a 41-year-old patient with Fontan palliation who developed nephrotic range proteinuria and had a kidney biopsy, which showed glomerular hypertrophy with segmental glomerulosclerosis around vascular poles, suggesting adaptive focal segmental glomerulosclerosis. This case provides further understanding of kidney dysfunction due to cyanotic congenital heart disease and shows the need for attention in the management for prevention of progression to end-stage renal disease and in the selection of renal replacement therapy.

Original languageEnglish
Pages (from-to)354-358
Number of pages5
JournalCEN case reports
Issue number3
Publication statusPublished - 2021 Aug 1


  • Adaptive FSGS
  • Cyanotic congenital heart disease
  • Cyanotic nephropathy
  • Kidney biopsy

ASJC Scopus subject areas

  • General Medicine


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