RT-176
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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
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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
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Abstract
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The differential diagnosis between primary Brugada’s syndrome (BS)
and a form secondary to an initial right ventricular dysplasia cardiomyopathy (RVDC) is a major challenge in
clinical arrhythmology today, because the arrhythmic risk stratification and the therapeutic approach to these
different clinical pictures could be extremely divergent.
We analyzed, in the present paper, our own experience of 64 subjects belonging to 9 different families. Baseline
clinical characteristics of this patient population has been considered. Furthermore, the role of different non
invasive and invasive diagnostic procedures have been evaluated.
In particular, the role of Echo and NMR imaging and the utility of intravenous flecainide drug testing in screening
patients to be submitted to PES, has been evaluated. Flecainide testing has been usefull in the evaluation of
family members of patients with the BS, but side effects, such as proarrhythmic events (ventricular arrhythmias
and QRS widening) were frequent. Furthermore the test was not reproducible in the same patients, some days
later.
In conclusion, evaluation of the personal and family history of such patients is mandatory and more useful before
the clinical evaluation and the non invasive evaluation is performed. Echo and NMR are useful in assessing the
presence of an underlying organic diseases, such as features of RVDC serial 24-hour Holter monitoring can
document frequently ectopic ventricular beats (EVBs) in RVDC cases, while rarely in BS.
PES, in members selected by non invasive methods, seems to be the appropriate methodology to select the most
adequate therapy in this condition, mainly for the asymptomatic patients, being the therapy with ICD well defined
for symptomatic patients. The place of genetic screening is not completely understood, at this point and probably
should be reserved to asymptomatic patients, without inducible arrhythmias and a typical Brugada’s ECG pattern.
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Key Words
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