†These authors contributed equally.
Academic Editor: Stefan Peters
Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia
(ARVC/D) is a hereditary disease characterized by fibrofatty infiltration of the
right ventricular myocardium that predisposes affected patients to malignant
ventricular arrhythmias, dual-chamber cardiac failure and sudden cardiac death
(SCD). The present study aims to investigate the risk of detrimental
cardiovascular events in an Asian population of ARVC/D patients, including the
incidence of malignant ventricular arrhythmias, new-onset heart failure with
reduced ejection fraction (HFrEF), as well as long-term mortality.
Methods and Results: This was a territory-wide retrospective cohort
study of patients diagnosed with ARVC/D between 1997 and 2019 in Hong Kong. This
study consisted of 109 ARVC/D patients (median age: 61 [46–71] years; 58%
male). Of these, 51 and 24 patients developed incident VT/VF and new-onset HFrEF,
respectively. Five patients underwent cardiac transplantation, and 14 died during
follow-up. Multivariate Cox regression identified prolonged QRS duration as a
predictor of VT/VF (p
