Mitochondrial electron transport chain deficiency, cardiomyopathy, and long-term cardiac transplant outcome

Pediatr Transplant. 2012 May;16(3):265-8. doi: 10.1111/j.1399-3046.2011.01635.x. Epub 2012 Jan 17.

Abstract

Organ transplantation in multisystemic mitochondrial cytopathies is usually not performed because of perceived untoward complications. We report three patients with demonstrated oxidative phosphorylation defects and dilated cardiomyopathy who underwent cardiac transplant. All three patients tolerated immunosuppression medications and have had an excellent long-term outcome. Our results suggest that with proper patient selection in this population, cardiac transplantation is feasible and can have good outcomes.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cardiomyopathies / pathology*
  • Child
  • Child, Preschool
  • Electron Transport
  • Female
  • Heart Transplantation / methods*
  • Humans
  • Immunosuppressive Agents / pharmacology
  • Male
  • Mitochondrial Diseases / metabolism
  • Mitochondrial Diseases / therapy*
  • Muscle, Skeletal / pathology
  • Outcome Assessment, Health Care
  • Oxidative Phosphorylation
  • Retrospective Studies
  • Spectrophotometry / methods
  • Time Factors
  • Treatment Outcome

Substances

  • Immunosuppressive Agents