Bibliogr. s. S44

ARVD/C is a genetic disorder of cardiac muscle that leads to replacement of myocytes with fibrofatty connective tissue. The disease primarily involves the right ventricle and leads to development of ventricular arrhythmias. The incidence of ARVD is about 0.02% in general population. The first clinical signs of ARVD are usually clinically apparent in young adults (under the age of 50). It accounts for a large number of sudden cardiac deaths in the young. ARVD is the most difficult type of cardiomyopathy to diagnose due to variability of symptoms and diagnostic difficulties despite use of both non-invasive and invasive methods. A number of clinical tests are employed to state the diagnosis of ARVD, including medical history review, electrocardiogram (ECG), echocardiography, right ventricular angiography, genetic testing and histological examination. The diagnosis of ARVD is based on a combination of major and minor criteria - so called revised criteria published in Circulation in 2010. Diagnostic imaging is a major factor in diagnosing ARVD, although each imaging method has its disadvantages. Cardiac MRI is currently considered the gold standard in noninvasive diagnostics of ARVD. MRI allows confirmation of global or regional RV akinesia, dyskinesia, or dyssynchrony, as determined by threshold values. On the other, hand the results of MRI alone are not sufficient to diagnose ARVD and other criteria must also be taken into account.