Among the other cases of SCA in this age group, 80% of patients with arrhythmogenic right ventricular dysplasia have ECG abnormalities, as do high proportions of patients with long-QT and Brugada syndromes and dilated cardiomyopathy [98,102-104].
Uhl's anomaly, arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD/C) and right ventricular outflow tract ventricular tachycardia (RVOT VT) involve pathogenic changes in the right ventricular (RV) myocardium.
Fontaine et al (2) described an entity, called arrhythmogenic right ventricular dysplasia, which was characterized, by localized deficiency or fibrofatty tissue replacement of right ventricular myocardium.
Etiology of sudden cardiac death in people [less than or equal to]35 years of age Hypertrophic cardiomyopathy 36% Coronary anomalies 19% Cardiac mass 10% Aortic rupture 5% Tunneled left anterior descending (LAD) 5% Aortic stenosis 4% Myocarditis 3% Dilated cardiomyopathy 3% Arrhythmogenic right ventricular dysplasia 3% Mitral valve prolapse 2% Coronary artery disease 2% Other 6% Table 2.
In our experience, similarity index was found significantly lower than normal in patients affected by congenital LOTS (7), in patients with arrhythmogenic right ventricular dysplasia (ARVD) and VTs (8), and in patients with myocardial infarction.