Effectiveness of High-dose Spironolactone Therapy in a Patient with Recurrent Protein-losing Enteropathy after the Fontan Procedure

Intern Med. 2016;55(12):1611-4. doi: 10.2169/internalmedicine.55.6303. Epub 2016 Jun 15.


Protein-losing enteropathy (PLE) is a rare and life-threatening complication that occurs after the Fontan procedure. We herein report the case of an 11-year-old Japanese boy who developed PLE six times after undergoing the Fontan procedure. High-dose spironolactone therapy has been effective for 2 years. His high level of serum aldosterone decreased to a nearly normal range and spironolactone may have a diuretic and anti-inflammatory potential.

Publication types

  • Case Reports

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use*
  • Child
  • Diuretics / therapeutic use*
  • Fontan Procedure / adverse effects*
  • Humans
  • Japan
  • Male
  • Protein-Losing Enteropathies / drug therapy*
  • Protein-Losing Enteropathies / etiology*
  • Spironolactone / therapeutic use*


  • Anti-Inflammatory Agents
  • Diuretics
  • Spironolactone