What is the arrhythmogenic dysplasia of the right ventricle?

The

heart is a four -mounted organ for pumping blood made up of muscles. In the arrhythmogenic dysplasia of the right ventricle (AVRD), the right chamber of the heart is primarily affected, where its muscle part is gradually replaced by fibrofatts with tissues. The left chamber can also be affected, but to a much lesser extent. Arrhythmogenic dysplasia/cardiomyopathy of the right ventricle (AVRD/C) is a heart disease inherited by an autosomal dominant way that predisposes the affected individual to excessive heart rate, fainting due to sudden loss of consciousness and death. During the clinical examination, the affected individual may present with non -specific symptoms related to heart disorders such as the distension of jugular vein, enlarged liver and swelling.

diagnosis of arrhythmogenic dysplasia of the right ventricle is determined by the filling of two main criteria or one main and two minor criteria, as the small criteria that can be obtained through the importPositive history and combination of non -invasive and invasive diagnostic tests. Non -invasive testing includes an electrocardiogram, a heart calculated tomographic scanning, a heart magnetic resonance imaging, echocardiography and holter monitoring. Invasive testing includes angiography of the right ventricle, electrophysiological testing and endomyocardial biopsy of the right ventricle. The main criteria include serious global or segmental dilation of the right ventricle, localized aneurysm in the right chamber, fibrofatta replacing the heart muscles, extension of QR located on right precordial wires and the presence of the disease confirmed in operation in the immediate family member. Smaller criteria include a slight global or segmental dilation of the right ventricle, reverse t waves located on real predordial wires, Block Block Chamber, and a family history of sudden death for less than 35 lethal in an immediate family member.

Managing persons with arrhythmogenic dysplasia of the right ventricle is IndivaIdualized and aimed at preventing excessive fast heart rate, fainting due to sudden loss of consciousness and death. Their prevention can be performed using antiarrhythmic drugs such as amiodarone and sotalol, implanted cardioverter-defibrillators and education. Implant cardioverter-defibrillators are considered to be high-risk individuals such as those who are intolerant or do not respond to antiarrrhythmic drugs, those who have been resuscitated, and persons with a family history of sudden cardiac arrest. Education about the risk of sudden death should include not only affected individuals but also their family members.

Regular screening of affected individuals and molecular genetic testing of immediate family members is also important. Screening is performed every year or more often depending on the symptoms using an electrocardiogram, echocardiogram or cardiac magnetic resonance imaging. Genetic testing reduces complications and death of early diagnosis and treatment.

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