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Arrhythmogenic Right Ventricular Cardiomyopathy

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[[Image:epsilon_wave.png|thumb|ECG with an epsilon wave in V1]]
[[Image:arvdhart.png|thumb| A section throughout the heart of an ARVC patient. (A) Transmural fatty replacement of the right ventricular free wall. (B) Myocardial atrophy is confined to the right ventricle and substantially spares the interventricular septum as well as the left ventricular free wall. <cite>Corrado</cite> Reproduced with permission from BMJ Publishing Group Ltd. ]]
'''Arrhythmogenic Right Ventricular Cardiomyopathy''', (ARVC, or ARVD: Arrhythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Most commonly the right ventricle apex and outflow tract are involved. However the left ventricle can be affected totoo.<cite>Corr</cite>
As a result of the fatty replacement and fibrosis, ventricular arrhythmias are common in this disease and can lead to palpitations, syncope and sudden death. At more advanced ages right ventricular pump failure can occur.
The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.<cite>McKenna1994</cite>
ARVC is a progressive disease. The '''incidence''' is estimated to be 1:3.000-1:10.000. Manifestations are usually seen in teenagers. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different chromosomal defects mutations have been described, most often with autosomal dominant 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).{| class="wikitable" width="400px"!Major diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy<cite>McKenna1994<An [http:/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<www.arvc.ca/li><li>Localized right ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging)<pdg/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 the ECG|-|<ul><li>(major) Epsilon wave or localized prolongation (>110ms) of help in assessing 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; risk 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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| style="width:300px" | '''Major'''
| style="width:300px" |'"''Minor'"''
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<li>Diagnostic terminology for original criteria: This diagnosis is fulfilled by the presence of 2 major, or 1 major plus 2 minor criteria or 4 minor criteria from different groups. Diagnostic terminology for revised criteria: definite diagnosis: 2 major or 1 major and 2 minor criteria or 4 minor from different categories; borderline: 1 major and 1 minor or 3 minor criteria from different categories; possible: 1 major or 2 minor criteria from different categories.</li>
<li><sup>&lowast;</sup> Hypokinesis is not included in this or subsequent definitions of RV regional wall motion abnormalities for the proposed modified criteria.</li>
<li><sup>&dagger;</sup> A pathogenic mutation is a DNA alteration associated with ARVC/D that alters or is expected to alter the encoded protein, is unobserved or rare in a large non–ARVC/D control population, and either alters or is predicted to alter the structure or function of the protein or has demonstrated linkage to the disease phenotype in a conclusive pedigree. E.g.: in TMEM43, DSP, PKP2, DSG2, DSC2, JUP.</li>
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