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Canadian Journal of Cardiology

Heart Transplant Indications, Considerations, and Outcomes in Fontan Patients: Age-Related Nuances, Transplant Listing, and Disease-Specific Indications

Published:February 28, 2022DOI:https://doi.org/10.1016/j.cjca.2022.02.019

      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.

      Résumé

      De nos jours, les patients qui ont subi l’intervention de Fontan meurent ou doivent subir une transplantation cardiaque pendant leur enfance dans 5 à 10 % des cas ou avant l’âge de 40 ans dans environ 50 % des cas. La transplantation cardiaque (TC) peut être pratiquée avec succès chez certains enfants et adultes qui ont subi l’intervention de Fontan et qui viennent à présenter une insuffisance circulatoire, quelle qu’en soit la cause. Par exemple, dans le cadre de la plus grande expérience monocentrique portant sur la TC postérieure à l’intervention de Fontan, le taux de survie à 1 an des personnes ayant subi une transplantation était de près de 90 % chez les enfants et de 80 % chez les adultes suivis. On peut s’attendre à ce que l’entéropathie avec perte de protéines et la bronchite plastique se résolvent après la transplantation, et les données limitées dont nous disposons donnent à penser que les patients atteints d’une maladie du foie associée à la circulation de Fontan qui survivent à la TC devraient voir leur fonction hépatique s’améliorer. La TC ne permet pas de remédier facilement à l’échec rapide de l’intervention de Fontan, c’est-à-dire moins de 12 mois après sa réalisation, et l’aiguillage tardif ou l’absence d’aiguillage des patients adultes vers les services de transplantation restent problématiques. On sait très peu de choses sur le nombre de patients qui ne sont pas aiguillés vers les services de transplantation, qui ne sont pas retenus comme candidats à la TC au terme d’une évaluation de leur admissibilité ou qui meurent dans l’attente d’une TC. Des données quantitatives sont nécessaires pour se faire une idée plus complète de la question des TC chez les patients ayant subi l’intervention de Fontan et pour mieux cibler nos efforts en matière d’amélioration de la qualité. Les révisions récentes de la priorisation des inscriptions au Canada tiennent compte spécifiquement du cas des patients ayant subi l’intervention de Fontan et visent à pallier le fait que les critères d’inscription en fonction du statut des patients ne sont pas adaptés au contexte des cardiopathies congénitales. Le recours à la transplantation chez les enfants à haut risque en amont de l’intervention de Fontan, la création de centres de transplantation ayant la capacité de prendre en charge les adultes atteints de cardiopathies congénitales et possédant l’expertise nécessaire pour pratiquer une transplantation combinée cœur-foie, au besoin, et l’amélioration des options et des critères d’assistance monoventriculaire mécanique applicables chez les adultes et les enfants peuvent contribuer à limiter la mortalité précoce parmi les patients en attente de transplantation.

      Graphical abstract

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