Brugada by any other name?

The article by Remme et al., and the editorial comment by Antzelevitch, fortuitously again forgot that the syndrome known worldwide as the Brugada syndrome had been described by Nava, Martini and Thiene more than 4 years before Brugada. There is autoptical evidence in the medical literature that this syndrome is related to some form of right heart disease (which does not mean typical right ventricular cardiomyopathy/ dysplasia), and to organic conduction disturbance. Despite this evidence, these authors insist that their patients are affected only by a functional disorder related to mutant SCN5A genes, the prevalence of which is very low in this syndrome despite the initial enthusiasm (and does not exclude an associated organic heart disease). These authors presumed to have identified 37 patients with ventricular fibrillation not related to organic heart disease, according to their extensive and rigorous investigations. They failed, however, to make a diagnosis of dilated cardiomyopathy and right ventricular cardiomyopathy/dysplasia in two patients submitted to autopsy. It is reasonable to assume that other patients in this series will have similar pathological substrates. This reported evidence demonstrates the problem of the sensitivity and specificity of invasive and non-invasive investigations, and of inter-observer variability and experience in the diagnosis of heart disease. We need more than the ECG (and other available diagnostic tools) to make a diagnosis of this complex syndrome, as well as of the underlying pathology and electrophysiological mechanisms. It must be emphasized that there is no simple single feature typical of the syndrome and no typical ECG. Different degrees of right bundle branch block, left axis deviation, and prolonged PR interval, have so often been described in this disease, as