Heart transplantation represents a valuable therapeutic option for patients with congenital heart disease and end-stage heart failure. We report the case of a 15-year-old patient with situs inversus and additional complex congenital malformations of the heart who underwent several previous palliative operations. Orthotopic cardiac transplantation with several technical modifications was performed successfully. At 18 months post-operatively the patient is free from rejection and doing well in New York Heart Association (NYHA) Class I status.
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