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J Arrhythm. 2017 Dec 21;34(1):11-22. doi: 10.1002/joa3.12021. eCollection 2018 Feb.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) in clinical practice.

Author information

1
Faculty of Medicine Newcastle University Newcastle UK.
2
Second Department of Cardiology Laboratory of Cardiac Electrophysiology "Evangelismos" General Hospital of Athens Athens Greece.
3
Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular disease Department of Cardiology Tianjin Institute of Cardiology Second Hospital of Tianjin Medical University Tianjin China.
4
Department of Anaesthesia and Intensive Care Faculty of Medicine Chinese University of Hong Kong Hong Kong China.
5
Li Ka Shing Institute of Health Sciences Faculty of Medicine Chinese University of Hong Kong Hong Kong China.
6
Department of Medicine and Therapeutics Faculty of Medicine Chinese University of Hong Kong Hong Kong China.
7
School of Life Sciences Chinese University of Hong Kong Hong Kong China.
8
The Jockey Club School of Public Health and Primary Care Faculty of Medicine The Chinese University of Hong Kong Hong Kong China.
9
Department of Pathology Rigshospitalet University Hospital of Copenhagen Copenhagen Denmark.
10
Norwich Medical School University of East Anglia Norwich UK.
11
Royal Brompton Hospital and Imperial College London London UK.

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited myocardial disease characterized by fibro-fatty replacement of the right ventricular myocardium, and associated with paroxysmal ventricular arrhythmias and sudden cardiac death (SCD). It is currently the second most common cause of SCD after hypertrophic cardiomyopathy in young people <35 years of age, causing up to 20% of deaths in this patient population. This condition has a male preponderance and is more commonly found in individuals of Italian and Greek descent. To date, there is no single diagnostic test for ARVC/D and the diagnosis is made based on clinical, electrocardiographic, and radiological findings according to the Revised 2010 Task Force Criteria. In this review, we will discuss the mainstay treatment which includes pharmacotherapy, implantable cardioverter-defibrillator insertion for abortion of sudden cardiac death, and in the advanced stages of the disease cardiac transplantation.

KEYWORDS:

arrhythmogenic right ventricular cardiomyopathy; arrhythmogenic right ventricular dysplasia

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