Neth Heart J. 2026 Nov 10. doi: 10.1007/s12471-026-02095-x. Online ahead of print.

ABSTRACT

Arrhythmias are common in transthyretin amyloid cardiomyopathy (ATTR-CM) and contribute substantially to morbidity at all disease stages. The arrhythmogenic substrate-reflecting diffuse amyloid infiltration, atrial myopathy, autonomic dysfunction, and conduction system involvement-differs fundamentally from that in other cardiomyopathies. Prospective rhythm monitoring reveals a burden substantially higher than previously recognised and predominantly asymptomatic, with bradyarrhythmias and atrial fibrillation (AF) dominating and sustained ventricular arrhythmias remaining rare.This review examines key aspects of arrhythmia management in ATTR-CM. Conduction system disease is frequently encountered, and pacing strategies-including conduction system pacing-are discussed in the context of progressive infiltrative disease. We address the rationale for lifelong anticoagulation in AF irrespective of CHA2DS2-VA-score, and emerging considerations for anticoagulation in selected sinus rhythm patients with impaired atrial function. Rhythm control strategies are reviewed in light of extensive atrial substrate, high AF recurrence rates, and the absence of randomised trial data. The role of implantable cardioverter-defibrillators is examined, with emphasis on the lack of proven survival benefit for primary prevention. Finally, we explore disease-modifying therapies and their potential to attenuate the arrhythmogenic substrate. Overall, structured surveillance, rhythm control, and individualised device decisions form the cornerstones of modern arrhythmia management in ATTR-CM.

PMID:43007391 | DOI:10.1007/s12471-026-02095-x