Prevalence and predictors of bradyarrhythmias requiring Permanent Pacing
in patients with Anderson-Fabry disease.
Abstract
Introduction: Bradyarrhythmias are an established red flag for storage
cardiac conditions including Anderson-Fabry disease (AFD). The
prevalence of bradyarrhythmias requiring a pacemaker (PM) and their
timing in AFD is unresolved. We evaluated prevalence and predictors of
PM requirement in a large AFD cohort, investigating the occurrence of
bradyarrhythmias as initial versus late manifestation. Methods: we
retrospectively evaluated 82 consecutive AFD patients referred to our
multidisciplinary referral centre from 1994 to 2020 with a median follow
up of 6.9 years, identifying those requiring pacing. Univariable
analysis was performed to identify cardiac features associated with PM
implantantion. Results: Five of 82 (6%) AFD patients required PM
implantation (5/39, i.e. 13% of those with cardiac involvement), always
in the context of advanced cardiomyopathy. In none, bradyarrhythmias
were the presenting feature. Indications included sick sinus syndrome in
3 patients, advanced atrio-ventricular block in 2 patients. QRS
prolongation during follow up strongly correlated with the onset of
bradyarrhythmias. Conclusions: Severe bradyarrhythmias are relatively
frequent in patients with AFD cardiomyopathy, but do not represent a
mode of presentation, occurring late in the disease course and always in
the context of advanced cardiac involvement. Monitoring QRS variations
over time may help to identify patients requiring pacing.