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Canadian Journal of Cardiology

Trends and Outcomes of Patients With Amyloid Cardiomyopathy Listed for Heart Transplantation

      Abstract

      Background

      Heart transplantation in patients with amyloid cardiomyopathy (ACM) has been historically underused owing to the risk of amyloid recurrence.

      Methods

      Using data from the United Network for Organ Sharing database on patients listed for single-organ heart transplant between 2010 and 2019, we evaluated trend in heart transplant and compared waitlist mortality and graft survival between patients with ACM and dilated cardiomyopathy (DCM). Also, we evaluated for independent predictors of outcomes.

      Results

      Over the study period, 411 adult patients with ACM were added to the heart transplant waitlist. In the propensity-matched cohorts, the rates of waitlist mortality were significantly higher for ACM compared with DCM (hazard ratio [HR], 1.75; 95% confidence interval [CI], 1.16-2.65). Over the study period, 330 patients with ACM underwent heart transplant. The number of transplants increased from 22 in 2010 to 59 in 2019 (168% increase). The 5-year graft survival rate was, however, significantly worse for ACM (78%) compared with DCM (82%) (HR,1.46, 1.03-2.08). We identified 2 predictors of graft failure among patients with ACM: namely, renal failure requiring dialysis (HR, 5.4, 1.6-17) and previous history of malignancy (HR, 1.6, 1.0-28). Patients with ACM with neither risk factor had 5-year graft survivals of 82%, which is comparable with DCM (HR, 1.28, 0.90-1.91). On the other hand, patients with ACM and either risk factor had worse 5-year graft survivals of 62% (HR, 2.44, 1.39-4.28).

      Conclusions

      Increasing numbers of patients with ACM are undergoing heart transplants. Although patients with ACM experience higher waitlist mortality and worse graft survival compared with DCM, selecting carefully screened ACM patients may result in improved outcomes following heart transplant.

      Résumé

      Contexte

      La transplantation cardiaque chez les patients atteints de cardiomyopathie amyloïde (CMA) a été historiquement sous-utilisée en raison du risque de récidive amyloïde.

      Méthodes

      En utilisant les informations de la base de données du United Network for Organ Sharing sur les patients inscrits pour une transplantation cardiaque d'un seul organe entre 2010 et 2019, nous avons évalué la tendance en matière de transplantation cardiaque et comparé la mortalité des patients sur liste d'attente et la survie du greffon entre les patients atteints de CMA et ceux atteints de cardiomyopathie dilatée (CMD). De plus, nous avons évalué des prédicteurs indépendants menant à pronostic.

      Résultats

      Au cours de la période d'étude, 411 patients adultes atteints de CMA ont été ajoutés à la liste d'attente pour une transplantation cardiaque. Dans des cohortes appariées selon un score de propension, les taux de mortalité des patients sur liste d'attente étaient significativement plus élevés pour la CMA que pour la CMD (rapport de risque [RR], 1,75; intervalle de confiance [IC] à 95 %, 1,16-2,65). Au cours de la période d'étude, 330 patients atteints de CMA ont subi une transplantation cardiaque. Le nombre de greffes est passé de 22 en 2010 à 59 en 2019 (augmentation de 168 %). Le taux de survie du greffon à 5 ans était cependant significativement plus mauvais pour la CMA (78 %) par rapport à la CMD (82 %) (RR,1,46, 1,03-2,08). Nous avons identifié deux facteurs prédictifs d'échec de la greffe chez les patients atteints de CMA : à savoir, une insuffisance rénale nécessitant une dialyse (RR, 5,4, 1,6-17) et des antécédents de tumeur maligne (RR, 1,6, 1,0-28). Les patients atteints de CMA ne présentant aucun facteur de risque avaient une survie du greffon à cinq ans de 82 %, ce qui est comparable à la CMD (RR, 1,28, 0,90-1,91). D'autre part, les patients atteints de CMA et présentant l'un ou l'autre des facteurs de risque avaient une plus mauvaise survie du greffon à cinq ans, de 62 % (RR, 2,44, 1,39-4,28).

      Conclusions

      Un nombre croissant de patients atteints de CMA subissent une transplantation cardiaque. Bien que les patients atteints de CMA connaissent une mortalité plus élevée quand ils sont sur liste d'attente, et une survie du greffon plus faible que les patients atteints de CMD, une sélection des patients atteints de CMA après un examen rigoureux peut conduire à de meilleurs pronostics après une transplantation cardiaque.
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      Linked Article

      • Heart Transplantation for Cardiac Amyloidosis: The Need for High-Quality Data to Improve Patient Selection
        Canadian Journal of CardiologyVol. 38Issue 8
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          Recent years have witnessed tremendous advances in the management of patients with cardiac amyloidosis. Important developments include improved awareness of the disease and how to recognize it, better diagnostic techniques, and—perhaps most significantly—new and more effective therapies to improve outcomes.1 Such advances have occurred for both amyloidosis disease subtypes that predominantly cause cardiac involvement; light-chain (AL) amyloidosis, a hematologic malignancy caused by clonal plasma cell proliferation, resulting in extracellular deposition of misfolded immunoglobulin light-chains, and transthyretin amyloidosis (ATTR), caused by misfolding of the hepatically derived transport protein transthyretin (TTR), resulting from either a mutation in the TTR gene (variant or hereditary ATTR [ATTRh]) or an age-related disorder occurring in the absence of a gene mutation (ATTR wild-type [ATTRwt]).
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