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

Alana S Golden1, Yuk M Law, Hillary Shurtleff

  • 1Division of Pediatric Neurology, Seattle Children's Hospital and University of Washington, Seattle, WA 98105, USA.

Pediatric Transplantation
|January 18, 2012
PubMed

Insights

Cardiac transplantation is feasible for patients with mitochondrial cytopathies and dilated cardiomyopathy. Careful patient selection leads to excellent long-term outcomes, challenging previous concerns about complications.

Area of Science:

  • Cardiology
  • Genetics
  • Transplantation Medicine

Background:

  • Multisystemic mitochondrial cytopathies often involve cardiac complications like dilated cardiomyopathy.
  • Organ transplantation is typically contraindicated in these patients due to presumed risks.

Purpose of the Study:

  • To evaluate the feasibility and outcomes of cardiac transplantation in patients with mitochondrial cytopathies and dilated cardiomyopathy.

Main Methods:

  • Retrospective case series of three patients with confirmed oxidative phosphorylation defects and dilated cardiomyopathy who underwent cardiac transplantation.
  • Monitoring of tolerance to immunosuppression and long-term follow-up.

Main Results:

  • All three patients successfully tolerated standard immunosuppression regimens.
  • Excellent long-term outcomes were observed in all transplanted patients.

Conclusions:

  • Cardiac transplantation can be a viable option for carefully selected patients with multisystemic mitochondrial cytopathies and dilated cardiomyopathy.
  • This approach can lead to favorable long-term results, contrary to prior expectations.

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