Contemporary Outcomes in Patients with Cardiac Amyloidosis Undergoing Heart Transplantation

Abstract

Purpose Cardiac amyloidosis causes significant morbidity and mortality, leading to restrictive cardiomyopathy, heart failure, and death. Many patients are diagnosed late when irreversible amyloid fibril deposition has occurred. Heart transplantation may be considered in such patients. Historically, heart transplant outcomes were worse in cardiac amyloid patients compared with other types of heart failure, in part due to the systemic nature of the disease. However, effective therapies, particularly for light chain (AL) amyloidosis, have emerged, and disease remission is now possible. We aimed to assess whether transplant outcomes for cardiac amyloidosis are better in the contemporary era. Methods We studied cardiac amyloid patients seen during 2004-2017 at the Stanford University and Kaiser Permanente Santa Clara medical centers who underwent heart transplantation. We examined pre-transplant characteristics and post-transplant outcomes in this group compared with the overall transplant population at our centers. Results During the study period, 31 patients (13 with AL amyloidosis and 18 with transthyretin (ATTR) amyloidosis) underwent heart transplantation. ATTR patients were older, more likely to be male, had worse baseline renal function, and had longer waitlist times compared with AL patients and the overall transplant population. Post-transplant, there were no differences in post-operative bleeding, renal failure, infection, rejection, or malignancy. There was no significant difference in the overall survival between cardiac amyloid patients and the overall population (median follow up: 1462 days; Figure 1). Conclusion This single-center, retrospective study reports detailed, long-term heart transplantation outcomes in a large cohort of cardiac amyloid patients. In carefully selected patients with cardiac amyloidosis, heart transplantation can be an effective therapeutic option with outcomes similar to those transplanted for other causes of heart failure.