page_title: Arrhythmogenic cardiomyopathy
section_title: Ventricular arrhythmias
hierarchical_section_title: Arrhythmogenic cardiomyopathy / Ventricular arrhythmias
caption_attribution_description: English: Monomorphic ventricular tachycardia originating from the right ventricular outflow tract. This particular patient had no response to en:adenosine 18mg fast IV push, and broke soon after an amiodarone IV push was given. The patient was eventually diagnosed with en:arrhythmogenic right ventricular dysplasia (ARVD). His baseline EKG had epsilon waves, which are characteristic of ARVD. The patient also had family history of sudden death of males in their early 20s (brother and an uncle) and echocardiographic findings consistent with ARVD.
context_page_description: Arrhythmogenic cardiomyopathy, arrhythmogenic right ventricular dysplasia, or arrhythmogenic right ventricular cardiomyopathy, is an inherited heart disease.
  ACM is caused by genetic defects of the parts of heart muscle known as desmosomes, areas on the surface of heart muscle cells which link the cells together. The desmosomes are composed of several proteins, and many of those proteins can have harmful mutations.
  The disease is a type of non-ischemic cardiomyopathy that primarily involves the right ventricle, though cases of exclusive left ventricular disease have been reported. It is characterized by hypokinetic areas involving the free wall of the ventricle, with fibrofatty replacement of the myocardium, with associated arrhythmias often originating in the right ventricle. The nomenclature ARVD is currently thought to be inappropriate and misleading as ACM does not involve dysplasia of the ventricular wall. Cases of ACM originating from the left ventricle lead to the abandonment of the name ARVC.
context_section_description: Ventricular arrhythmias due to ACM typically arise from the diseased right ventricle.  The type of arrhythmia ranges from frequent premature ventricular complexes (PVCs) to ventricular tachycardia (VT) to ventricular fibrillation (VF).
  While the initiating factor of the ventricular arrhythmias is unclear, it may be due to triggered activity or reentry.
  Ventricular arrhythmias are usually exercise-related, suggesting that they are sensitive to catecholamines.  The ventricular beats typically have a right axis deviation.  Multiple morphologies of ventricular tachycardia may be present in the same individual, suggesting multiple arrhythmogenic foci or pathways.
  Right ventricular outflow tract (RVOT) tachycardia is the most common VT seen in individuals with ACM.  In this case, the EKG shows a left bundle branch block (LBBB) morphology with an inferior axis.
caption: Monomorphic ventricular tachycardia originating from the right ventricular outflow tract
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