Heart transplantation in cardiac amyloidosis

Abstract

"Cardiac amyloidosis" is the term commonly used to reflect the deposition of abnormal protein amyloid in the heart. This process can result from several different forms, most commonly from light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, which in turn can represent wild-type (ATTRwt) or genetic form. Regardless of the origin, cardiac involvement is usually associated with poor prognosis, especially in AL amyloidosis. Although several treatment options, including chemotherapy, exist for different forms of the disease, cardiac transplantation is increasingly considered. However, high mortality on the transplantation list, typical for patients with amyloidosis, and suboptimal post-transplant outcomes are major issues. We are reviewing the literature and summarizing pros and cons of listing patients with amyloidosis for cardiac or combine organ transplant, appropriate work-up, and intermediate and long-term outcomes. Both AL and ATTR amyloidosis are included in this review.

Cardiac amyloidosis: An update on diagnosis and treatment

“…Contemporary series of patients undergoing heart transplant followed by stem cell transplant showed that outcomes are almost comparable to heart transplant for other indications, with a 5-year survival rate of approximately 65%. 74 Future therapies for AL. There is an AL amyloid-directed monoclonal antibody designed to remove amyloid fi brils from affected organs and is currently undergoing clinical trials.…”

“…Patients with the ATTRm mutation V122I have been treated with heart transplant alone, with the thought process that again, due to the indolent nature of amyloid deposition, concomitant liver transplant may not be needed. 74 Thus far, 6 patients with this mutation have undergone successful transplant at our institution with heart alone, 1 of whom is 9 years posttransplant without any recurrent amyloid in the allograft. Patients with the T60A mutation that causes both polyneuropathy and cardiomyopathy require combined heart and liver transplant.…”

“…Patients with the T60A mutation that causes both polyneuropathy and cardiomyopathy require combined heart and liver transplant. 74 Are there other therapies being studied to clear amyloid deposits and reverse organ dysfunction? Extracellular deposits of amyloid fi brils, regardless of precursor protein, contain common elements such as calcium, glycosaminoglycans, and an SAP component.…”

Mechanical circulatory support for cardiac amyloidosis

“…Recent advances in the treatment of AL and ATTR amyloid cardiomyopathy include the advent of proteasome inhibitors for AL amyloid, and mRNAi therapy and tafamidis for ATTR amyloid. However, many patients often progress to end‐stage heart failure and in such patients, if the systemic manifestations are controlled, heart transplantation can improve survival …”

Hereditary systemic transthyretin amyloidosis: a clinical case and an opinion on the problem

“…Гистологическое исследование тканей при окраске конго красным позволяет выявить специфически дов лечения амилоидоза в этом случае считается трансплантация сердца, после которой, однако, возможен рецидив заболевания, поэтому дополнительно проводится трансплантация печени [14]. При тяжелой нефропатии рассматривается трансплантация печени и почек [15].…”