Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis

Eur J Pediatr. 1992 Mar;151(3):200-3. doi: 10.1007/BF01954384.


Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectionosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amylopectin / analysis
  • Biopsy
  • Cardiomyopathies / complications*
  • Cardiomyopathies / pathology
  • Cardiomyopathy, Dilated / etiology*
  • Cardiomyopathy, Dilated / pathology
  • Glycogen Storage Disease Type IV / complications*
  • Glycogen Storage Disease Type IV / pathology
  • Glycogen Storage Disease Type IV / surgery
  • Humans
  • Infant
  • Liver / chemistry
  • Liver / pathology
  • Liver Transplantation*
  • Male
  • Microscopy, Electron
  • Myocardium / chemistry
  • Myocardium / pathology


  • Amylopectin