Cardiac amyloidosis (CA), encompassing transthyretin amyloidosis (ATTR) and light-chain amyloidosis (AL), poses significant challenges in managing advanced heart failure (HF). This chapter explores the role of mechanical circulatory support (MCS) devices and heart transplantation (HT) in treating CA, emphasizing recent advancements that have improved outcomes. MCS devices, including durable left ventricular assist devices (LVADs) and temporary mechanical support, have limited utility in CA due to restrictive cardiac physiology and systemic involvement. While durable LVADs are rarely used, they may serve as a bridge to HT in selected ATTR cases. Temporary MCS devices can stabilize cardiogenic shock but are often constrained by high complication rates and poor outcomes. HT remains the only curative option for end-stage HF in CA. For AL CA, achieving a durable hematologic response to chemotherapy is critical to prevent recurrent amyloid deposition post-transplant. Combining HT with autologous stem cell transplantation (ASCT) has significantly improved survival, with five-year rates exceeding 60% in carefully selected patients. ATTR CA patients demonstrate excellent post-HT outcomes, with five-year survival rates surpassing 70%. In hereditary ATTR (ATTRv), combined heart and liver transplantation may be required, whereas isolated HT is typically sufficient for wild-type ATTR (ATTRwt). Advances in disease-modifying therapies, including transthyretin stabilizers and gene-silencing agents, have further enhanced survival and reduced recurrence. Recent policy changes have improved prioritization for CA patients requiring HT, reducing waitlist mortality and wait times. Post-transplant outcomes in CA patients now parallel those in non-CA populations, with comparable survival rates and complications. As systemic therapies evolve and patient selection criteria refine, integrating MCS and HT with disease-targeted treatments provides a multidisciplinary strategy for optimizing outcomes in advanced HF secondary to CA.

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Management of Advanced Heart Failure in Cardiac Amyloidosis

  • Rabea Asleh,
  • Offer Amir

摘要

Cardiac amyloidosis (CA), encompassing transthyretin amyloidosis (ATTR) and light-chain amyloidosis (AL), poses significant challenges in managing advanced heart failure (HF). This chapter explores the role of mechanical circulatory support (MCS) devices and heart transplantation (HT) in treating CA, emphasizing recent advancements that have improved outcomes. MCS devices, including durable left ventricular assist devices (LVADs) and temporary mechanical support, have limited utility in CA due to restrictive cardiac physiology and systemic involvement. While durable LVADs are rarely used, they may serve as a bridge to HT in selected ATTR cases. Temporary MCS devices can stabilize cardiogenic shock but are often constrained by high complication rates and poor outcomes. HT remains the only curative option for end-stage HF in CA. For AL CA, achieving a durable hematologic response to chemotherapy is critical to prevent recurrent amyloid deposition post-transplant. Combining HT with autologous stem cell transplantation (ASCT) has significantly improved survival, with five-year rates exceeding 60% in carefully selected patients. ATTR CA patients demonstrate excellent post-HT outcomes, with five-year survival rates surpassing 70%. In hereditary ATTR (ATTRv), combined heart and liver transplantation may be required, whereas isolated HT is typically sufficient for wild-type ATTR (ATTRwt). Advances in disease-modifying therapies, including transthyretin stabilizers and gene-silencing agents, have further enhanced survival and reduced recurrence. Recent policy changes have improved prioritization for CA patients requiring HT, reducing waitlist mortality and wait times. Post-transplant outcomes in CA patients now parallel those in non-CA populations, with comparable survival rates and complications. As systemic therapies evolve and patient selection criteria refine, integrating MCS and HT with disease-targeted treatments provides a multidisciplinary strategy for optimizing outcomes in advanced HF secondary to CA.