Abstract
Arrhythmogenic right ventricular dysplasia is an inherited fatal cardiomyopathy causing fat and connective tissue infiltration of right ventricle. In advanced disease, it may also involve left ventricle. Heart failure, arrhythmias and sudden death are main clinical components. Many different genetic mutations can cause this cardiomyopathy. All mutations lead to the formation of the disease are not yet defined. Basis of pathogenetic event is corruption of physical connection as well as the electrical conduction between cardiomyocytes due to these genetic reasons. Genetical inheritance is frequently autosomal dominant but incomplete. Because of the many different phenotypes, there may be great heterogenity of clinical manifestations in same family. Diagnostic criteria are renovated lately. Current cardiac imaging techniques for the diagnosis is gradually progressing. A combination of several tests should be used for diagnosis. Early and accurate diagnosis will save lives and improve the patient’s quality of life. In this paper, after mentioning the features and diagnosis of ARVD, differential diagnosis of ARVD from other diseases which may cause fatty infiltration of heart, arrhythmias and sudden death will be focused.
Highlights
In recent years, with the contribution of the developments on diagnostic methods in patients with heart failure, major progress has been made in determining some of the rare and deadly diseases
There are many diseases that mimic some features of the arrhythmogenic right ventricular dysplasia (ARVD) in current clinical practice
Two dimensional echocardiography is not recommended for the the evaluation of right ventricle (RV) volume, 3D TTE or 3D TEE is suggested method
Summary
With the contribution of the developments on diagnostic methods in patients with heart failure, major progress has been made in determining some of the rare and deadly diseases. Accuracy of differential diagnosis will accelerate the treatment process and dramatically reduce the morbidity and mortality of the disease This genetic cardiomyopathy involves mainly the right ventricle (RV) and rarely left ventricle (LV). Major and minor diagnostic criteria obtained from six categorical classes defined by 1-global/regional structural and functional change, 2-tissue characterisation, 3repolarization abnormalities, 4-depolarization/conduction abnormalities, 5-arrhythmias and 6-family history [7]. Limitations of the MRI assessment are being dependent of the sensitivity and specificity of practitioners, artifacts due to cardiac arrhythmias, bad images, limited use of MRI and presence of intracardiac devices It is not a method which is possible to applicate everywhere and everyone [13]. If diagnosis is still not definite, diagnosis can be made by electrophysiological study and endocardial voltage mapping and typical myocardial scar can be diagnosed at early stage [15]
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