How to Treat Heart Failure in Patients with Cardiac Amyloidosis

Abstract

Amyloidosis is a multisystem disease characterized by deposition of fibrillar proteins in different tissues, the heart being one of the sites of amyloid deposition. Cardiac amyloidosis may present as a restrictive syndrome with symptoms of heart failure (HF). Treatment includes prognosis-modifying medications in HF; their optimization, however, is a challenge due to the frequent adverse effects in this population. Renin-angiotensin-aldosterone system blockade should be carried out using low doses because, due to autonomic dysfunction, these drugs may cause hypotension and fatigue. The use of diuretics is necessary to maintain euvolemic status and optimized preload; however, due to the ventricular restriction present in cardiac amyloidosis, these medications should be used with caution. In patients with amyloidosis who progress to advanced HF, there are particularities in relation to indications for heart transplantation or long-term ventricular assist devices. The objective of this article is to review the treatment alternatives for HF in patients with cardiac amyloidosis.Amyloidosis is a multisystem disease characterized by the deposition of fibrillar proteins with specific structural characteristics and a beta-pleated shape. The deposition of these proteins in the heart and the involvement of the autonomic nervous system make it challenging to manage heart failure (HF) in these patients. Given this scenario, This article aims to demonstrate how to manage HF in patients with cardiac amyloidosis. Approach to treatment of heart failure in patients with cardiac amyloidosisData on HF treatment in patients with amyloidosis is lacking in randomized clinical trials. Therapeutic recommendations are based on expert opinions and experiences.The deposition of fibrillar proteins in the myocardium leads to a reduction in the ventricular cavity, with consequent restricted filling, initially culminating in HF with preserved ejection fraction and finally HF with reduced

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“…João David de Souza Neto and Juan Alberto Cosquillo Mejía have been in charge, actively participating in the Technical Chambers of the Ministry of Health and elaborating guidelines, including Brazilian guidelines for HF, heart transplantation, and circulatory assistance mechanics. [2][3][4][5][6][7][8][9][10][11] (Figure 3) On January 4, 1999, a 39-year-old carpenter named Antônio Pereira de Moura became the first patient to have his heart transplanted at HM, marking the dawn of a new era in cardiology in Ceará. (Figure 4A) A 36-year-old woman donated the organ after a diagnosis of brain death in HM's respiratory ICU; with both recipient and donor placed side by side, the procedure was carried out with a successful ischemia time of less than 90 minutes.…”

Diabetic Cardiomyopathy and Heart Failure

“…However, antidiabetic drugs have different effects in patients with HF, and clinicians must prioritize drugs that are safe in this setting and reduce HF-related events. 60 Poor glycemic control unquestionably contributes to the increased risk of HF in patients with diabetes, with HF risk correlating with glycated hemoglobin (HbA1c) levels. 61 Prior to the current era, intensive glucose-lowering therapies largely failed to reduce the risk of incident HF and could even exacerbate the risk of HF -e.g., thiazolidinediones, sulfonylureas, certain dipeptidyl peptidase-4 (DPP-4) inhibitors, and, potentially, insulin and glucagon (GLP-1) agonists.…”

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“…O autocuidado compreende a tomada de decisão e manutenção de hábitos de vida saudáveis e gerenciamento de sintomas [5][6] . Envolve o desenvolvimento de habilidades pelo indivíduo para atividades como mensuração do peso e de edemas; atividade física; assiduidade às consultas; dieta; medicamentos; reconhecimento dos sinais e sintomas de descompensação e manejo dos sinais e sintomas e confiança para tomadas de decisões frente à IC [5][6] .…”

“…Pacientes com IC que apresentam um autocuidado mais eficaz comprovadamente têm melhor qualidade de vida e menores taxas de readmissão e mortalidade [4][5] . Apesar disso, já foi evidenciado que apenas entre 7% e 18% das pessoas com IC referem comportamentos adequados de autocuidado 7 .…”