Myocardial Disease: Difference between revisions

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===Arrythmic cardiomyopathy: Arrhythmogenic Right Ventricular Cardiomyopathy===
===Arrythmic cardiomyopathy: Arrhythmogenic Right Ventricular Cardiomyopathy===
[[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 too. 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 failure can occur.  
Arrhythmogenic Right Ventricular Cardiomyopathy, (ARVC, or ARVD: Arrhythmogenic Right Ventricular Disease, also AC arrhythmogenic Cardiomyopathy) 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. 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 failure can occur.  


ARVC is a progressive disease, and its incidence is estimated to be 1:3.000-1:10.000. The disease usually manifests at adolescence. Although the diagnosis is more often confirmed in athletes, physical activity is not thought to have a causal relationship with the disease. ARVC can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant inheritance. One unique form of ARVC, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.  
ARVC is a progressive disease, and its incidence is estimated to be 1:3.000-1:10.000. The disease usually manifests at adolescence. Although the diagnosis is more often confirmed in athletes, physical activity is not thought to have a causal relationship with the disease. ARVC can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant inheritance. One unique form of ARVC, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.