Abstract
Cardiac amyloidosis (CA) is considered to be associated with an increased risk of sudden cardiac death (SCD) due to ventricular tachyarrhythmias and electromechanical dissociation. However, current arrhythmic risk stratification and the role of an implantable cardioverter-defibrillator (ICD) for primary prevention of SCD remains unclear. This article provides a narrative review of the literature on electrophysiological abnormalities in the context of ventricular arrhythmias in patients with CA and the role of ICD in terms of survival benefit in this group of patients.
Highlights
Amyloidosis is a rare systemic disease characterized by the extracellular deposition of pathological insoluble fibrillar protein, known as amyloid, within various organs
We performed a narrative review, rather than a systematic review of the literature, that focuses on arrhythmic sudden cardiac death and the role of implantable cardioverter-defibrillator (ICD) in patients with Cardiac amyloidosis (CA)
DataPreliminary are available on the arrhythmic risk stratification six patients with amyloidosis in our group showed a normal result in all of of risk the usual approach in these patients is secondary prevention or extrapolation them, as well as normal values of baroreflex sensitivity (BRS); this research is currently ongoing
Summary
Amyloidosis is a rare systemic disease characterized by the extracellular deposition of pathological insoluble fibrillar protein, known as amyloid, within various organs (mainly the heart and kidneys). The most common types of cardiac amyloidosis (CA) are caused by immunoglobulin-derived light chains (AL) and the precursor protein transthyretin (ATTR). A TTR tetramer stabilizer, is the most extensively studied medication that showed the reduction in all-cause mortality and hospitalization rates in ATTR CA, especially if applied in early stages of the disease [5]. Novel treatment vastly improved survival in AL and ATTR cardiac amyloidosis, cardiovascular events account for more than two-thirds of fatal casualties in both groups [6]. Electromechanical dissociation is thought to be the most common cause of SCD in patients with cardiac amyloidosis; ventricular arrhythmias and conduction abnormalities are common [8]. Little is known about risk factors for ventricular tachyarrhythmia’s as a cause of SCD in patients with amyloidosis and cardiac involvement. It remains unclear whether ICD prevents SCD in these patients
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