14th International Congress
THE "NEW FRONTIERS"
OF ARRHYTHMIAS 2000

Jan. 29 - Feb. 5, 2000
Marilleva, Trento, Italy

RT-173

Further evidence that “Brugada syndrome” can be due to arrhythmogenic right ventricular cardiomyopathy with disease locus in chromosome 14q24.3

Andrea Nava, Barbara Bauce, Alessandra Rampazzo**, Bortolo Martini***, Michela Muriago, Sergio Cannas***, Cristina Basso* Gaetano Thiene*, Gian Antonio Danieli**.
Departments of Cardiology, *Pathology and **Biology, University of Padua Medical School, Padua, ***Department of Cardiology, Thiene Hospital, Italy

Introduction

The presence of ST segment elevation in the right precordial leads in non ischaemic patients has been considered for many years a natural variant of the ECG called “early repolarisation” with no pathologic significance¹.

In 1988 this ECG aspect was described for the first time in patients who had died suddenly or had an abortive sudden death and who were diagnosed with arrhythmogenic right ventricular cardiomyopathy^2,3. Thus the ST segment elevation was interpreted as a consequence of the intraventricular conduction disturbance due to the presence of fibrous-fatty tissue in the right ventricular myocardium. In these patients the intraventricular conduction was so fragmented that it always produced late potentials.

Later Brugada and Brugada collected many cases of patients with this ECG pattern who had had aborted sudden death. In their series no structural heart disease was found and late potential were always absent⁴.

In recent years this syndrome has been widely studied and many cases with or without structural heart disease have been reported. Moreover an alteration of ionic channels genetically determined has been demonstrated^5-12.

A family proved to be affected by arrhythmogenic right ventricular cardiomyopathy is reported in which one family member presents the Brugada’s ECG pattern. The modality of appearance of the ECG alteration and the association with chromosome 14 lead us to make different hypotheses about the pathogenesis of this disease.