Background: In recent years, rapid advances in cardiac surgery and changes in attitude towards patients with cognitive disability have led to these patients receiving cardiac transplantation.
Method: This is a retrospective report describing the experience of four patients with Down Syndrome who received heart transplantation in a single institution.
Results: Anthracycline-induced cardiomyopathy was the most common cause of heart failure in this group (3/4). Two patients were bridged to transplantation, one by using a combination of extra-corporeal membrane oxygenation and biventricular assist device and the other by using a durable implantable left ventricular assist device. All the four patients are alive with the longest surviving patient 17 years after transplantation. Against strong hypothetical predictions, we observed no propensity for the development of post-transplant infections or lymphoproliferative disorders.
Conclusion: Down Syndrome should not be the sole contraindication to heart transplantation. The decision for transplantation should be on a case-by-case basis provided adequate social support is in place.
Keywords: Down syndrome; PTLD; heart transplantation; immunosuppression and quality of life; mechanical circulatory support.
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