Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males

The Brugada syndrome displays an autosomal dominant mode of transmission with low penetrance. Despite equal genetic transmission of the disease, the clinical phenotype is 8 to 10 times more prevalent in males than in females. The basis for this intriguing sex-related distinction is unknown. The pres...

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Bibliographic Details
Published in:Circulation (New York, N.Y.) N.Y.), 2002-10, Vol.106 (15), p.2004-2011
Main Authors: DI DIEGO, José M, CORDEIRO, Jonathan M, GOODROW, Robert J, FISH, Jeffrey M, ZYGMUNT, Andrew C, PEREZ, Guillermo J, SCORNIK, Fabiana S, ANTZELEVITCH, Charles
Format: Article
Language:English
Subjects:
Action Potentials - drug effects
Animals
Arrhythmias, Cardiac - diagnosis
Arrhythmias, Cardiac - etiology
Arrhythmias, Cardiac - physiopathology
Biological and medical sciences
Cardiac dysrhythmias
Cardiology. Vascular system
Cells, Cultured
Culture Techniques
Dogs
Female
Heart
Heart - physiology
Kinetics
Male
Medical sciences
Pericardium - drug effects
Pericardium - physiology
Phenotype
Pinacidil - pharmacology
Sex Factors
Syndrome
Terfenadine - pharmacology
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Summary:The Brugada syndrome displays an autosomal dominant mode of transmission with low penetrance. Despite equal genetic transmission of the disease, the clinical phenotype is 8 to 10 times more prevalent in males than in females. The basis for this intriguing sex-related distinction is unknown. The present study tests the hypothesis that the disparity in expression of the Brugada phenotype is a result of a more prominent I(to)-mediated action potential notch in the right ventricular (RV) epicardium of males versus females. We studied epicardial tissue slices, arterially perfused wedge preparations, and dissociated epicardial myocytes isolated from male and female canine hearts. RV epicardium action potential phase 1 amplitude was 64.8+/-2.0% of that of phase 2 in males compared with 73.8+/-4.4% in females (P
ISSN:0009-7322
1524-4539
DOI:10.1161/01.cir.0000032002.22105.7a