Cardiac Transplantation Outcomes in Patients with Amyloid Cardiomyopathy

Objective Amyloid cardiomyopathy (ACM) is a progressive and life-threatening disease characterized by abnormal proteinaceous deposits within the cardiac extracellular space. The most common forms of ACM are caused by immunoglobulin derived light chains (AL) and transthyretin (TTR). Although the management for each entity is different, heart transplant (HT) remains the definitive treatment for patients who progress to end stage heart failure. To date there is no consensus regarding the usage of heart transplantation in patients with ACM, and studies have shown mixed results. In this study, we perform a contemporary multi-center analysis evaluating post HT survival in patients with ACM. Methods We conducted a multi-center analysis of 62588 adult HT recipients captured in the United Network for Organ Sharing (UNOS) registry from 1987-2018. Patients were characterized as ACM or Non-ACM. Baseline characteristics were obtained, and summary characteristics were calculated. Outcomes of interest included post-transplant survival, infection, treated rejection, and the ability to return to work. Unadjusted associations between ACM and non-ACM survival were determined using the Kaplan-Meier estimations and confounding was addressed using multivariable Cox proportional hazards models. Results 398 patients with a diagnosis of ACM were identified. ACM patients were older (61 vs 52 p= Conclusion In this analysis of HT in ACM, ACM was associated with a higher likelihood of post HT mortality. No differences were observed in episodes of treated rejection, hospitalization for infection or likelihood to return to work for income. Further analysis is warranted to establish the mechanism of higher mortality in ACM patients.