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

Ka Hou Christien Li, George Bazoukis, Tong Liu, Guangping Li, William KK Wu, Sunny Hei Wong, Wing Tak Wong, Yat Sun Chan, Katharina Wassilew, Vassilios S Vassiliou, Gary Tse

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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.
Original languageEnglish
Pages (from-to)11–22
Number of pages12
JournalJournal of Arrhythmia
Volume34
Issue number1
Early online date21 Dec 2017
DOIs
Publication statusPublished - Feb 2018

Keywords

  • arrhythmogenic right ventricular cardiomyopathy
  • arrhythmogenic right ventricular dysplasia

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