Successful cardiac transplantation in Barth syndrome--single-centre experience of four patients

Pediatr Transplant. 2007 May;11(3):327-31. doi: 10.1111/j.1399-3046.2006.00629.x.


We describe four patients with Barth syndrome who have undergone successful orthotopic heart transplantation. Patients are one, seven, 12.5 and 14.7 yr post-transplantation. One episode of severe infection occurred. Renal dysfunction and coronary allograft vasculopathy do not appear accelerated over non-Barth patients. Despite withholding purine synthesis inhibitors, these patients have not demonstrated an increased rate of rejection.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Cardiomyopathies / etiology*
  • Cardiomyopathies / surgery*
  • Child
  • Child, Preschool
  • Genetic Diseases, X-Linked / complications*
  • Heart Transplantation*
  • Humans
  • Infant
  • Male
  • Metabolism, Inborn Errors / complications*
  • Syndrome
  • Treatment Outcome