Heart Transplant Indications,Considerations, and Outcomes in Fontan Patients: Age-Related Nuances,Transplant Listing,and Disease-Specific Indications |
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| Affiliation: | 1. Department of Pediatrics, University of Toronto, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada;2. Division of Cardiac Surgery, University of Toronto, Labatt Family Heart Centre, The Hospital for Sick Children, Peter Munk Cardiac Center, Toronto, Ontario, Canada;3. Division of Cardiology, Toronto General Hospital, Peter Munk Cardiac Centre, Toronto, Ontario, Canada;4. Department of Medicine, University of Toronto, Toronto, Ontario, Canada;1. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA;2. Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA;1. Department of Psychiatry and Behavioural Neurosciences, McMaster University, Hamilton, Ontario, Canada;2. School of Rehabilitation Science, McMaster University, Hamilton, Ontario, Canada;3. Child Health Evaluative Science, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada;4. Factor-Inwentash Faculty of Social Work, University of Toronto, Toronto, Ontario, Canada;5. Transplant and Regenerative Medicine Centre, The Hospital for Sick Children, Toronto, Ontario, Canada;1. Jewish General Hospital, MAUDE Unit, McGill University, Montréal, Québec, Canada;2. Toronto Adult Congenital Heart Disease Program, Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada;3. University Health Network, Toronto, Ontario, Canada;4. Ohio State University Medical Center, Columbus, Ohio, USA;5. St. Boniface General Hospital, University of Manitoba, Winnipeg, Manitoba, Canada;6. Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada;7. McGill University Health Centre, Montréal, Québec, Canada;1. Department of Pediatrics (Neurology), The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada;2. Department of Pediatrics (Neurology), BC Children’s Hospital and University of British Columbia, Vancouver, British Columbia, Canada;3. Division of Neurology, Vancouver Stroke Program, University of British Columbia, Vancouver, British Columbia, Canada |
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| Abstract: | In the current era, 5%-10% of Fontan patients die or need a transplant in childhood, and approximately 50% will experience the same fate by age 40 years. Heart transplant (HTx) can be successful for selected children and adults with Fontan circulatory failure of any mechanism, with a 1-year post-transplant survival rate approaching 90% in children and 80% in the largest single-centre adult Fontan HTx experience. Protein-losing enteropathy and plastic bronchitis can be expected to resolve post-transplant, and limited data suggest patients with Fontan-associated liver disease who survive HTx can expect improvement in liver health. Early Fontan failure, within 12 months of Fontan completion, is not easily rescued by HTx, and late referrals and failure to refer adult patients remain problematic. Very little is known about the numbers of patients who are not referred, are turned down following assessment for HTx, or die on the waiting list—numbers that are needed to understand the complete picture of HTx in the Fontan population and to identify where best to focus quality-improvement efforts. Recent revisions to listing prioritization in Canada with considerations specific to the Fontan population aim to mitigate the fact that the status-listing criteria are not tailored to the congenital heart population. Transplanting high-risk children prior to Fontan completion, developing adult congenital heart disease transplant centres with expertise that can also offer combined heart-liver transplant when appropriate, and improving single-ventricle mechanical support options and criteria for both adults and children may help mitigate the early post-listing mortality. |
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