Cardiac Transplant, Great Ormond Street Hospital, London, UK.
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.