Transplantation in the single ventricle population

The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair. Progress in surgical palliative strategies has impacted upon the present composition of the population, with growing numbers of hypoplastic left heart syndrome patients and those with late physiology failure following Fontan completion. Multiple prior surgeries, immunological sensitivity and multi-organ involvement impart high peri-operative risk but can be mitigated in part by careful pre-operative planning by a dedicated multi-disciplinary team addressing issues such as planning of concurrent reconstructive surgery, minimizing the post-operative effect of collaterals, timely harvesting, oversizing of donor organs to minimize graft failure, and strategies to address anticipated post-operative elevation in pulmonary vascular resistance. Determining optimal timing for transplant in these patients remains unclear, but understanding the risk of alternative surgical options can help guide decision making with regards to listing.