Arrhythmic Manifestations of Cardiac Amyloidosis: Challenges in Risk Stratification and Clinical Management

Abstract

Amylodiosis is a systemic disease characterized by extracellular deposits of insoluble amyloid in various tissues and organs. Cardiac amyloidosis is a frequent feature of the disease, causing a progressive, restrictive type of cardiomyopathy, and is associated with adverse clinical outcomes and increased mortality. The typical clinical presentation in patients with cardiac amyloidosis is heart failure (HF) with preserved ejection fraction. Most patients present with typical symptoms and signs of HF, such as exertional dyspnea, pretibial edema, pleural effusions and angina pectoris due to microcirculatory dysfunction. However, patients may also frequently encounter various arrhythmias, such as atrioventricular nodal block, atrial fibrillation and ventricular tachyarrhythmias. The management of arrhythmias in cardiac amyloidosis patients with drugs and devices is often a clinical challenge. Moreover, predictors of life-threatening arrhythmic events are not well defined. This review intends to give a deepened insight into the arrhythmic features of cardiac amyloidosis by discussing the pathogenesis of these arrhythmias, addressing the challenges in risk stratification and strategies for management in these patients.

Prognostic implications of premature ventricular contractions and non-sustained ventricular tachycardia in light-chain cardiac amyloidosis

“…Since CA has gained more clinical recognition in the field of heart failure, 22 , 23 various arrhythmias ranging from brady- and tachyarrhythmias accompanying this disease have also been increasingly addressed. 5 In particular, VAs and their potential link to adverse clinical outcomes have been a matter of concern. However, identification of patients with CA who may benefit from ICD therapy remains to be a challenge.…”

“… 1 , 2 There are two main forms of disease, namely light chain amyloidosis ( A L) and transthyretin amyloidosis (ATTR), both of which provide a substrate for electro-mechanical remodeling 3 , 4 and various arrhythmias. 5 Compared with patients with the ATTR subtype, those with AL-CA often develop complex ventricular arrhythmias (VA) and have a poorer prognosis. 1 , 3 It was reported that 5–27% of AL-CA patients present with a non-sustained ventricular tachycardia (NSVT), 6 , 7 and premature ventricular contractions (PVCs) were found in 72% patients.…”

Rare-variant collapsing and bioinformatic analyses for different types of cardiac arrhythmias in the UK Biobank reveal novel susceptibility loci and candidate amyloid-forming proteins

“… 1 Both acquired and common and rare genetic and risk factors exist for cardiac arrhythmias. 1 , 2 Cardiac amyloidosis is a rare disease that may manifest various arrhythmias, such as atrioventricular nodal block, atrial fibrillation, and ventricular tachyarrhythmias. 2 Amyloidosis is characterized by extracellular deposits of amyloid in various organs.…”

“… 1 , 2 Cardiac amyloidosis is a rare disease that may manifest various arrhythmias, such as atrioventricular nodal block, atrial fibrillation, and ventricular tachyarrhythmias. 2 Amyloidosis is characterized by extracellular deposits of amyloid in various organs. 2 Cardiac amyloidosis with cardiomyopathy is a frequent feature of amyloidosis.…”

“… 2 Amyloidosis is characterized by extracellular deposits of amyloid in various organs. 2 Cardiac amyloidosis with cardiomyopathy is a frequent feature of amyloidosis. Patients with cardiac amyloidosis frequently encounter various arrhythmias.…”