IMR Press / RCM / Volume 23 / Issue 7 / DOI: 10.31083/j.rcm2307231
Open Access Original Research
A Territory-Wide Study of Arrhythmogenic Right Ventricular Cardiomyopathy Patients from Hong Kong
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1 Cardiovascular Analytics Group, Laboratory of Cardiovascular Physiology, Hong Kong, China
2 School of Data Science, City University of Hong Kong, Hong Kong, China
3 Faculty of Medicine, Newcastle University, NE1 7RU Newcastle upon Tyne, UK
4 Aston Medical School, Aston University, B4 7ET Birmingham, UK
5 Arrhythmia Unit, Department of Cardiovascular Medicine, First Affiliated Hospital of Xi'an Jiaotong University, 710061 Xi'an, Shaanxi, China
6 Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Department of Cardiology, Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University, 300211 Tianjin, China
7 State Key Laboratory of Agrobiotechnology (CUHK), School of Life Sciences, Chinese University of Hong Kong, Hong Kong, China
8 Centre for Safe Medication Practice and Research, Department of Pharmacology and Pharmacy, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, China
9 School of Pharmacy, University College London, WC1E 6BT London, UK
10 Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong Hospital Authority, Hong Kong, China
11 Department of Pathology, Hong Kong Children’s Hospital, Hospital Authority, Hong Kong, China
*Correspondence: (Gary Tse); (Qingpeng Zhang)
These authors contributed equally.
Academic Editor: Stefan Peters
Rev. Cardiovasc. Med. 2022, 23(7), 231;
Submitted: 17 November 2021 | Revised: 12 May 2022 | Accepted: 16 May 2022 | Published: 24 June 2022
(This article belongs to the Special Issue Arrhythmogenic Cardiomyopathy: Diagnosis and Therapy)
Copyright: © 2022 The Author(s). Published by IMR Press.
This is an open access article under the CC BY 4.0 license.

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 < 0.05). Female gender, prolonged QTc duration, the presence of epsilon waves and T-wave inversion (TWI) in any lead except aVR/V1 predicted new-onset HFrEF (p < 0.05). The presence of epsilon waves, in addition to the parameters of prolonged QRS duration and worsening ejection fraction predicted all-cause mortality (p < 0.05). Clinical scores were developed to predict incident VT/VF, new-onset HFrEF and all-cause mortality, and all were significantly improved by machine learning techniques. Conclusions: Clinical and electrocardiographic parameters are important for assessing prognosis in ARVC/D patients and should in turn be used in tandem to aid risk stratification in the hospital setting.

arrhythmogenic right ventricular cardiomyopathy/dysplasia
heart failure
ventricular arrhythmias
Fig. 1.
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