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Pediatric heart transplantation is a curative treatment for DCM and CHD with excellent clinical mid-term results. However, further follow-up is necessary to evaluate long-term side effects of immunosuppressants. Donor shortage remains a problem.

Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two-ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.

We report a rare case of a successful cardiac transplantation in a patient suffering from cardiomyopathy and complex mitochondrial disease. The patient presented with severe heart failure and malignant ventricular arrhythmias requiring implantation of a defibrillator and advanced medical treatment. The patient was listed for urgent heart transplantation and received a donor heart after 36 days. One yr post-operatively, the patient has completely recovered.

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