A Territory-Wide Study of Arrhythmogenic Right Ventricular Cardiomyopathy Patients from Hong Kong

Ishan Lakhani, Jiandong Zhou, Sharen Lee, Ka Hou Christien Li, Keith Sai Kit Leung, Jeremy Man Ho Hui, Yan Hiu Athena Lee, Guoliang Li, Tong Liu, Wing Tak Wong, Ian Chi Kei Wong, Ngai Shing Mok, Chloe Miu Mak, Qingpeng Zhang, Gary Tse

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)

Abstract

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.

Original languageEnglish
Article number231
JournalReviews in Cardiovascular Medicine
Volume23
Issue number7
DOIs
Publication statusPublished - 2022
Externally publishedYes

Keywords

  • arrhythmogenic right ventricular cardiomyopathy/dysplasia
  • heart failure
  • mortality
  • ventricular arrhythmias

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