Open AccessFemale Predominance and Transmission Distortion in the Long-QT Syndrome
Author(s) -
Medea Imboden,
Heikki Swan,
Isabelle Denjoy,
Irene M. van Langen,
Päivi Johanna Latinen-Forsblom,
Carlo Napolitano,
Véronique Fressart,
G Breithardt,
Myriam Berthet,
Silvia G. Priori,
Bernard Hainque,
Arthur A.M. Wilde,
Eric SchulzeBahr,
Josué Feingold,
Pascale Guicheney
Publication year - 2006
Publication title -
new england journal of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 19.889
H-Index - 1030
eISSN - 1533-4406
pISSN - 0028-4793
DOI - 10.1056/nejmoa042786
Subject(s) - offspring , long qt syndrome , mendelian inheritance , medicine , non mendelian inheritance , allele , qt interval , sudden death , genetics , cardiology , biology , pregnancy , gene , mitochondrial dna
Congenital long-QT syndrome is a disorder resulting in ventricular arrhythmias and sudden death. The most common forms of the long-QT syndrome, types 1 and 2, are caused by mutations in the potassium-channel genes KCNQ1 and KCNH2, respectively. Although inheritance of the long-QT syndrome is autosomal dominant, female predominance has often been observed and has been attributed to an increased susceptibility to cardiac arrhythmias in women. We investigated the possibility of an unbalanced transmission of the deleterious trait.
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