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Porcari et al. Vessel Plus 2022;6:33 https://dx.doi.org/10.20517/2574-1209.2021.134 Page 5 of 14

Figure 1. Current management and grey areas in cardiac amyloidosis. Courtesy of Professor Rossana Bussani, MD, Institute of
Pathological Anatomy and Histology, University of Trieste, Italy. AL: Light chain amyloidosis; ACE-i/ARBs: angiotensin-converting
enzyme inhibitors/angiotensin receptor blockers; AF: atrial fibrillation; AV: atrio-ventricular; AVR: aortic valve replacement; BBs: beta
blockers; CA: cardiac amyloidosis, CRT: cardiac resynchronization therapy; DCCV: direct current cardioversion; hATTR: hereditary
transthyretin amyloidosis; HF: heart failure; ICD: implantable cardioverter defibrillator; LAA: left atrial appendage; LF-LG: low-flow low-
gradient; LVEF: left ventricular ejection fraction; MRAs: mineral receptor antagonists; PM: pacemaker; RV: right ventricle; SCD: sudden
cardiac death; SR: sinus rhythm; TAVI: transcatheter aortic valve implantation; TOE: transesophageal echocardiography; VAs:
ventricular arrhythmias.

output . In addition, CA patients are prone to hypotension and electrical conduction abnormalities,
[21]
[2]
particularly atrio-ventricular (AV) blocks, which can be worsened by BBs . Particularly in AL-CA,
orthostatic hypotension is commonly reported due to dysfunction of the autonomic nervous system or
toxicity from chemotherapy agents . These medications might be better tolerated in patients with ATTR-
[21]
CA lacking significant autonomic neuropathy.

Available data about the use of HF medications in CA are controversial and derive from retrospective
studies [Table 1]. However, concerns about safety of BBs, angiotensin-converting enzyme inhibitors or
angiotensin receptor blockers (ACE-i/ARBs), and mineral receptor antagonists (MRAs) were evident in the
Transthyretin Amyloidosis Cardiomyopathy Clinical Trial (ATTR-ACT) as fewer than 30% of patients were
treated with these medications . In recent real-world reports, BBs were frequently prescribed with up to
[8]
40% of CA patients under therapy at first evaluation and well-tolerated in the absence of known
[25]
contraindications .
[26]
In a retrospective cohort of 99 patients with AL- and ATTR-CA followed for 16 months, BBs, ACE-i, and
MRAs were well tolerated in the absence of contraindications with fewer than 10% of patients experiencing
adverse effects attributable to these drugs (most frequently, hypotension and symptomatic bradycardia) .
[27]
Conversely, in a single-center, retrospective study including 480 ATTR-CA patients, the use of BBs and
ACE-i reduced survival in variant ATTR-CA and did not affected outcome in wild-type ATTR-CA at 41
months . In this study, patients on HF medications had a more severe cardiac involvement, thus
[28]
potentially having a worse outcome per se. Therefore, the safety, tolerability, and prognostic impact of anti-
neurohormonal drugs need to be tested in further studies, differentiating ATTR- from AL-CA. Meanwhile,