Transgenic Animal Models

  • Matteo Vatta
  • Zhao Yang
  • Jeffrey A. Towbin


Arrhythmogenic right ventricular cardiomyopathy (ARVC/D) is a paradigm shifting disorder that primarily affects the right ventricular (RV) myocardium early in the course of disease with later-onset left ventricular (LV) disease [1]. Clinically, the disease is classically characterized by ventricular arrhythmias of RV origin, as noted by ventricular tachycardia (VT) with a left bundle branch block (LBBB) morphology, commonly associated with syncope or sudden cardiac death (SCD) [2]. In addition, affected individuals may develop right heart failure and ultimately biventricular failure. In most cases, age of onset occurs in the teenage years or beyond, more often in males than females [3]. In the Veneto region of Italy, the disorder has been described as the most common cause of SCD in young, healthy, and athletic individuals, in contradistinction to that title given to hypertrophic cardiomyopathy (HCM) in the United States (US) [2, 4, 5]. In the US, ARVC/D is believed to be responsible for approximately 5% of unexplained cases of SCD in young athletic individuals while this number rises to 25%-30% in Italy [5].


Right Ventricular Sudden Cardiac Death Final Common Pathway Intercalate Disk Ventricular Cardiomyopathy 
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Copyright information

© Springer-Verlag Italia 2007

Authors and Affiliations

  • Matteo Vatta
    • 1
  • Zhao Yang
    • 1
  • Jeffrey A. Towbin
    • 1
  1. 1.Pediatric Cardiology, Texas Children’s HospitalBaylor College of MedicineHoustonUSA

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