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

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

RT-176

Accurate non invasive evaluation and genetic screening could help in differentiating congenital Brugada’s syndrome from the acquired one, secondary to right ventricular dysplasia cardiomyopathy

Franco Naccarella*, Giovannina Lepera, Fabio Iachetti, Antonella Bartoletti, Mauro Gatti, Mario Coluccini, Pietro Ticci***, Luigi Padeletti***, Andrea Nava**.
*Cardiology Department, Azienda USL della Citta di Bologna, Bologna, **Cardiology, University of Padova, ***Clinical Electrophysiology, Clinica Medica, University of Firenze, Italy

In recent papers, it has been proposed that Brugada’s syndrome (BS) might be due to genetically determined Na channels pathology, in the absence of an otherwise demonstrable heart disease^1-62.

Nevertheless, cases with minor anatomical pathological features of the right ventricle and infundibulum have been reported, with a negative genetic screening, for the typical chromosome localizations documented in right ventricular dysplasia cardiomyopathy (RVDC)^1,2. Furthermore, some cases that have displayed the typical Brugada ST T pattern, in the absence of an organic heart disease, later evolved into manifest RVDC⁴⁷. In other cases, with a clinical diagnosis of RVDC^46-48, a comcomitant Brugada ECG pattern has been shown. In these cases, an underlying concomitant Na channels pathology and RVDC chromosomes mutation can be postulated. Alternatively, a new chromosome localization for RVDC should be suspected. In these cases, a specific involvement of the high right ventricle or of the right infundibulum should be postulated in association with the typical localizations of the RVDC (apex, area behind the tricuspid valve, and infundibular area under pulmonary valves)^{20,26-32,34,44-47}.

Thus, both a primary form of BS without any sign of organic heart disease^{1-3,6,7,12,14,17} and a secondary form of the disease, due to an initial pattern of RVDC should be suspected^1,2,61,62.

TABLE I – Differential features in the Brugada’s syndrome and RVDC (modified from Brugada)

ECG 1, 2, 3=ECG aspects; Echo/angio=echocardiographic and angiographic aspects; NMR=NMR features; PET SCAN=positron emission tomography aspects.