Hereditary prolongation of QT interval . Study of two families

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...

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Bibliographic Details
Published in:British heart journal 1970-07, Vol.32 (4), p.505-509
Main Authors: Gale, G E, Bosman, C K, Tucker, R B, Barlow, J B
Format: Article
Language:English
Subjects:
Adolescent
Adrenergic beta-Antagonists - therapeutic use
Adult
Aged
Child
Child, Preschool
Deafness - congenital
Death, Sudden
Electrocardiography
Female
Genes, Dominant
Genes, Recessive
Heart Diseases - drug therapy
Heart Diseases - epidemiology
Heart Diseases - genetics
Humans
Infant
Male
Middle Aged
Pedigree
South Africa
Syncope - drug therapy
Syncope - epidemiology
Syncope - genetics
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Summary: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.
ISSN:0007-0769
1355-6037
DOI:10.1136/hrt.32.4.505