Arrhythmogenic Right Ventricular Cardiomyopathy: Difference between revisions

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Arrhythmogenic Right Ventricular Cardiomyopathy (view source)

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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.  
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).
'''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). An [arvc.ca/pdg/public.php?rep=arvc_cri online calculator] can help in assessing the risk in an individual patient. In 2009 these criteria were updated<cite>cox</cite><cite>Crit2010</cite>, see the table below.
{| class="wikitable" width="400px"
!Major diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy<cite>McKenna1994</cite>
|-
|<ul><li>Familial disease confirmed at necroscopy or surgery</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 can be diagnosed on the ECG
|-
|<ul>
<li>(major) Epsilon wave or localized prolongation (>110ms) of the QRS complex 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>
|}
In 2009 these criteria were updated<cite>cox</cite><cite>Crit2010</cite>, see the table below.


'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite>
'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite>
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