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Arrhythmogenic Right Ventricular Cardiomyopathy

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One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.
'''Diagnosis''' ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnostic criteria for the diagnosis of ARVC<cite>#McKenna1994</cite> (see table).{| class="wikitable" width="400px"!Major diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy<cite>McKenna1994<An [>|-|<ul><li>Familial disease confirmed at necroscopy or surgery<pdg/li><li>Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) LV impairment</li><li>Localized right ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging)</li><li>Severe segmental dilataion of the right ventricle</li><li>Fibrofatty replacement of myocardium on endomyocardial biopsy</li></ul>|-!Diagnostic criteria that public.php?rep=arvc_cri online calculator] can be diagnosed on help in assessing the ECG|-|<ul><li>(major) Epsilon wave or localized prolongation (>110ms) of the QRS complex risk in right precordial leads (V1-V3)</li><li>(minor) Inverted T waves in right precordial leads (V2 and V3) (people aged more than 12 yr; in absence of [[RBBB]]</li><li>(minor) [[Late potentials]] ([[SAECG|signal averaged ECG]])</li><li>(minor) Left bundle branch block type [[Ventricular Tachycardia|ventricular tachycardia]] (sustained and non-sustained) (ECG, [[Holter]], [[Exercise Testing|exercise testing]]</li><li>(minor) Frequent [[Ventricular Premature Beats|ventricular extrasystoles]] (more than 1000/24h) ([[Holter]])</li></ul>|}an individual patient. In 2009 these criteria were updated<cite>cox</cite><cite>Crit2010</cite>, see the table below.
'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite>

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