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Page 4 of 14 Porcari et al. Vessel Plus 2022;6:33 https://dx.doi.org/10.20517/2574-1209.2021.134

There are no defined indications to CRT in CA; HF guidelines?
PM-dependent patients and those with frequent RV pacing (> 40% of the time) might benefit Should CRT be offered based on HF symptoms and typical LBBB,
from CRT implantation. irrespective of LVEF?
Might para-hissian pacing be feasible in CA? Should it be preferred to
RV pacing?

AL: Light chain amyloidosis; ACE-i/ARBs: angiotensin-converting enzyme inhibitors/angiotensin receptor blockers; AF: atrial fibrillation; AS: aortic valve stenosis; ATTR: transthyretin amyloidosis; AV: atrio-
ventricular; AVR: aortic valve replacement; BBs: beta blockers; CA: cardiac amyloidosis; CAD: coronary artery disease; CABG: coronary artery bypass graft; CRT: cardiac resynchronization therapy; DCCV: direct
current cardioversion; HF: heart failure; ICD: implantable cardioverter defibrillator; LAA: left atrial appendage; LBBB: left bundle branch block; LF-LG: low-flow low-gradient; LVEF: left ventricular ejection fraction;
MRAs: mineral receptor antagonists; PCI: percutaneous coronary intervention; PM: pacemaker; RV: right ventricle; SCD: sudden cardiac death; SR: sinus rhythm; TAVI: transcatheter aortic valve implantation.

advanced stages of the disease .
[22]

AL and ATTR amyloidosis: different extracardiac clinical manifestations
Patients with AL amyloidosis frequently present with renal and autonomic nervous system involvement, but gastrointestinal and soft tissue involvement can

occur . Most patients have normal serum creatinine levels at diagnosis and progressive renal failure develops in ≈20% of cases. Proteinuria results from
[23]
amyloid deposition at the glomerular level and can evolve to the nephrotic range in 30%-50% of cases, sometimes leading to hypoalbuminemia requiring

supplementation. Autonomic neuropathy can present with symptomatic orthostatic hypotension of different magnitude, ranging from only associated with
exertion to severe enough to prevent the completion of ordinary activities.

Gastrointestinal involvement might cause weight loss, malabsorption, gastrointestinal bleeding, and development of motility disturbances. Hepatomegaly and
increased serum alkaline phosphatase levels suggest liver infiltration.

Extracardiac involvement in wtTTR amyloidosis predominantly consists in the presence of carpal tunnel syndrome (commonly with bilateral

[9]
[24]
manifestations) . Conversely, vTTR amyloidosis frequently manifests as sensorimotor polyneuropathy accompanied by autonomic symptoms .

METHODS
This is a narrative review dealing with the most frequent clinical questions and needs encountered in the management of patients with CA in everyday

practice. The content of each section addresses a specific topic providing information based on experts’ opinions supported by scientific literature.

GREY ZONES

Neurohormonal antagonists and supportive treatment
Peculiar pathophysiological conditions limit the introduction and up-titration of HF medications in CA. In detail, beta-blockers (BBs) are generally considered
contraindicated or poorly tolerated due to unfavorable hemodynamic effects of slowing the heart rate because of inability to adequately increase cardiac