Steven Hamilton, Harsh Patolia, Mazen Hanna
PURPOSE OF REVIEW: The management of end-stage heart failure in cardiac amyloidosis due to both light chain (AL-CA) and transthyretin (ATTR-CA) with heart transplantation has historically been limited by concerns about systemic progression after transplant. As a result, heart transplantation as a strategy was often restrictive and many patients were excluded. This review synthesizes contemporary evidence and outlines a practical framework to guide heart transplant evaluation and decision making in advanced disease. We emphasize evolving multidisciplinary selection principles.
RECENT FINDINGS: In the recent era (since the 2010s), multicenter experience has demonstrated that carefully selected patients with advanced AL-CA and ATTR-CA can achieve favorable outcomes after heart transplantation. Specifically, selected individuals with wild type transthyretin cardiac amyloidosis (ATTRwt-CA) may be appropriate candidates. For hereditary transthyretin cardiac amyloidosis (ATTRv-CA), isolated heart or combined heart liver transplantation (CHLT) can been considered, although modern transthyretin targeted therapies increasingly influence this decision. Patients with AL-CA may undergo transplantation alongside timely plasma cell directed treatment. Historically, outcomes for heart transplantation in patients with cardiac amyloidosis were poor. Improved patient selection as well as advances in plasma cell targeted therapy for AL-CA and transthyretin stabilizing or gene silencing treatments for ATTR-CA have reshaped expectations. The improved outcomes seen in more contemporary cohorts support heart transplantation as a viable option for selected patients with advanced disease.