RT Journal Article
SR Electronic
T1 Hereditary prolongation of QT interval
JF British Heart Journal
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP 505
OP 509
DO 10.1136/hrt.32.4.505
VO 32
IS 4
A1 G. E. Gale
A1 C. K. Bosman
A1 R. B. K. Tucker
A1 J. B. Barlow
YR 1970
UL http://heart.bmj.com/content/32/4/505.abstract
AB A syndrome has previously been recognized, which is characterized by recurrent episodes of loss of consciousness, some of which end fatally. The electrocardiogram in affected subjects shows prolongation of the QT interval.In the present study, 2 unrelated families with a total membership of 82 were investigated; 30 living subjects were examined and 20 were found to be affected. A further 14 members, 11 of whom died suddenly, were presumed from their histories to have been affected. The condition seems to be much more common, at least in South Africa, than the small number of previously reported cases would suggest.In contrast to the similar syndrome in which congenital deafness is also a feature and in which the disorder is transmitted in an autosomal recessive manner, analysis of the present data reveals an autosomal dominant inheritance with variable penetrance. The fundamental nature of the disorder remains unknown. Though treatment is generally unsatisfactory, beta-adrenergic blocking agents may be of value.