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Arrhythmogenic Right Ventricular Cardiomyopathy: Difference between revisions
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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 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. ]] | [[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 | '''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 too.<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. | 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. | ||
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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 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 | 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 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. | 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). 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. | '''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 [http://www.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. | ||
'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite> | '''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite> | ||