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Published on: July 20, 2022
Arrhythmic Risk Stratification in Cardiac Amyloidosis: A Review of the Current Literature
Eleonora Bonvicini1, Alberto Preda2, Chiara Tognola3
1Department of Cardiology, S. Chiara Hospital, 38122 Trento, Italy.
Insights
Cardiac amyloidosis, a protein-misfolding disease, causes heart failure and arrhythmias. This review examines arrhythmic risk stratification and therapeutic strategies for this growing condition.
Area of Science:
- Cardiology
- Cardiovascular Research
- Infiltrative Cardiomyopathies
Background:
- Cardiac amyloidosis is the leading infiltrative heart disease, stemming from misfolded protein deposition.
- It causes heart failure, conduction disorders, and potentially fatal arrhythmias like atrial fibrillation and ventricular arrhythmia.
- Early diagnosis and management strategies for cardiac amyloidosis-related arrhythmias remain challenging.
Purpose of the Study:
- To review current evidence on arrhythmic risk stratification in cardiac amyloidosis.
- To discuss available and emerging therapeutic strategies for managing arrhythmias in these patients.
- To highlight unresolved issues in diagnosis and treatment.
Main Methods:
- Literature review of recent studies on cardiac amyloidosis and arrhythmias.
- Analysis of data on electrophysiological disturbances, risk stratification tools, and treatment outcomes.
- Synthesis of evidence regarding novel therapies and their impact on arrhythmia prevention.
Main Results:
- Extreme bradycardia is a primary cause of arrhythmic death; ventricular arrhythmias occur even in early stages.
- Atrial impairment increases AF risk, often resistant to treatment, and leads to recurrent thromboembolic events.
- Increasing diagnoses due to population aging and improved imaging necessitate updated management guidelines.
Conclusions:
- Effective risk stratification and timely intervention are crucial for preventing sudden cardiac death in cardiac amyloidosis.
- Current novel therapies aim to improve patient outcomes but require further evaluation for arrhythmia prevention.
- Further research is needed to optimize diagnosis and treatment strategies for arrhythmias in cardiac amyloidosis.
Abstract:
Cardiac amyloidosis is the most frequent infiltrative disease caused by the deposition of misfolded proteins in the cardiac tissue, leading to heart failure, brady- and tachyarrhythmia and death. Conduction disorders, atrial fibrillation (AF) and ventricular arrhythmia (VA) significantly impact patient outcomes and demand recognition. However, several issues remain unresolved regarding early diagnosis and optimal management. Extreme bradycardia is the most common cause of arrhythmic death, while fast and sustained VAs can be found even in the early phases of the disease. Risk stratification and the prevention of sudden cardiac death are therefore to be considered in these patients, although the time for defibrillator implantation is still a subject of debate. Moreover, atrial impairment due to amyloid fibrils is associated with an increased risk of AF resistant to antiarrhythmic therapy, as well as recurrent thromboembolic events despite adequate anticoagulation. In the last few years, the aging of the population and progressive improvements in imaging methods have led to increases in the diagnosis of cardiac amyloidosis. Novel therapies have been developed to improve patients' functional status, quality of life and mortality, without data regarding their effect on arrhythmia prevention. In this review, we consider the latest evidence regarding the arrhythmic risk stratification of cardiac amyloidosis, as well as the available therapeutic strategies.
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