Arrhythmias in Patients with Cardiac Amyloidosis: A Comprehensive Review on Clinical Management and Devices

Abstract

Cardiac amyloidosis (CA) is a rare but potentially life-threatening disease in which misfolded proteins accumulate in the cardiac wall tissue. Heart rhythm disorders in CA, including supraventricular arrhythmias, conduction system disturbances, or ventricular arrhythmias, play a major role in CA morbidity and mortality, and thus require supplementary management. Among them, AF is the most frequent arrhythmia during CA hospitalizations and is associated with significantly higher mortality, while ventricular arrhythmias are also common and are usually associated with poor prognosis. Early diagnosis of potential arrythmias could be performed through ECG, Holter monitoring, and/or electrophysiology study. Clinical management of these patients is quite significant, and it usually includes initiation of amiodarone and/or digoxin in patients with AF, potential electrical cardioversion, or ablation in specific patients with indication, as well as initiation of anticoagulants in all patients, independent of AF and CHADS-VASc score, for potential intracardiac thrombus. Moreover, identification of patients with conduction disorders that could benefit from prophylactic pacemaker implantation and/or CRT as well as identification of patients with life-threatening ventricular arrythmias that could benefit from ICD could both increase the survival rates of these patients and improve their quality of life.

Keywords: arrhythmias; atrial fibrillation; cardiac amyloidosis; clinical management; devices; ventricular arrhythmias.

Figure 1

Proposed algorithm for permanent pacemaker implantation in patients with cardiac amyloidosis and arrythmias [20,31,37,51,52,53,58].

Figure 2

Proposed algorithm for implantable cardioverter-defibrillator (ICD) in patients with cardiac amyloidosis and arrythmias [20,37,54].

Figure 3

Clinical management for patients with cardiac amyloidosis and arrythmias [20,37,52,54].