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Arrhythmogenic Right Ventricular Cardiomyopathy: Difference between revisions
Arrhythmogenic Right Ventricular Cardiomyopathy (view source)
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[[Image:epsilon_wave.png|thumb|ECG with an epsilon wave in V1]] | [[Image:epsilon_wave.png|thumb|ECG with an epsilon wave in V1]] | ||
[[Image:arvdhart.png|thumb| A section | [[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. ]] | ||
''' | '''Arrhytmogenic Right Ventricular Cardiomyopathy''', (ARVC, or ARVD: Arrhythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Especially the right ventricle apex and outflow tract are involved. However the left ventricle can be affected to.<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 | 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> | 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 | ARVC is a progressive disease. The '''incidence''' is estimated to be 1:3.000-1:10.000. Manifestations are usually seen during the teens. 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 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. | 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 | '''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" | {| class="wikitable" width="400px" | ||
!Major diagnostic criteria for | !Major diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy<cite>McKenna1994</cite> | ||
|- | |- | ||
|<ul><li>Familial disease confirmed at necroscopy or surgery</li> | |<ul><li>Familial disease confirmed at necroscopy or surgery</li> | ||
<li>Severe dilatation and | <li>Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) LV impairment</li> | ||
<li> | <li>Localized right ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging)</li> | ||
<li>Severe segmental dilataion of the right ventricle</li> | <li>Severe segmental dilataion of the right ventricle</li> | ||
<li>Fibrofatty replacement of myocardium on endomyocardial biopsy</li> | <li>Fibrofatty replacement of myocardium on endomyocardial biopsy</li> | ||
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|- | |- | ||
|<ul> | |<ul> | ||
<li>(major) Epsilon wave or | <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) 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) [[Late potentials]] ([[SAECG|signal averaged ECG]])</li> | ||
<li>(minor) Left bundle branch block type [[Ventricular Tachycardia|ventricular tachycardia]] ( | <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> | <li>(minor) Frequent [[Ventricular Premature Beats|ventricular extrasystoles]] (more than 1000/24h) ([[Holter]])</li> | ||
</ul> | </ul> | ||
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'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite> | '''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite> | ||
*Medication: | *Medication: | ||
**anti- | **anti-arrhythmics: Sotalol better than Amiodarone. | ||
**ACE-inhibitors to prevent cardiac remodelling | **ACE-inhibitors to prevent cardiac remodelling | ||
*[[ICD]] implantation is recommended for the prevention of sudden cardiac death in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy. | *[[ICD]] implantation is recommended for the prevention of sudden cardiac death in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy. | ||