Association of secundum atrial septal defect with abnormalities of atrioventricular conduction or left axis deviation. Genetic study of 10 families.

British Heart Journal
R EmanuelM Hegde

Abstract

A genetic analysis was made of 10 families in which the propositi had a secundum atrial septal defect associated with abnormal atrioventricular conduction (first, second, or third degree heart block) or unexplained left axis deviation or a combination of these conduction disturbances. Diagnostic information was available on 51 (81%) of the first degree relatives. Three of the families appeared to be examples of a new syndrome which, with variable expression, was inherited as a non-sex-linked autosomal dominant. The main features were a secundum atrial septal defect; disease of the conducting tissue, which in some cases was progressive; unexplained left axis deviation; and unexpected death. These families did not seem to be examples of the Holt-Oram syndrome, for the upper limbs were clinically and radiologically normal in the 19 members examined. The importance of recognizing this syndrome is the occurrence of progressive disease of the conducting tissue and the risk of sudden death. When, therefore, unexplained left axis deviation or prolonged atrioventricular conduction is found in association with a secundum atrial septal defect all available relatives should be examined. In the remaining seven families there was only one af...Continue Reading

References

Jul 1, 1975·British Heart Journal·E EsscherM Michaëlsson
May 1, 1970·British Heart Journal·R Emanuel
May 6, 1974·The American Journal of Cardiology·B E VictoricaI H Gessner
Jul 1, 1974·British Heart Journal·C M MorgansG H Robb
Dec 1, 1973·British Heart Journal·D J Goodman, E W Hancock
Sep 1, 1974·The American Journal of Cardiology·K M HynesE R Giuliani
May 1, 1972·The American Journal of Cardiology·E C MathewsJ I Townsend
Jul 1, 1970·British Heart Journal·A McDonaldL McDonald
May 1, 1971·British Heart Journal·A McDonaldL McDonald
Sep 1, 1968·British Heart Journal·R EmanuelJ Somerville
Jan 1, 1967·British Heart Journal·R Amarasingham, H A Fleming
Mar 1, 1967·Circulation·J J NoraF C Fraser
Dec 25, 1958·The New England Journal of Medicine·R A CARLETONE W HANCOCK
Sep 1, 1961·British Heart Journal·M CAMPBELL, P E POLANI
Sep 1, 1961·British Heart Journal·G HOWITT
Oct 5, 1961·The New England Journal of Medicine·M H WEIL, B J ALLENSTEIN
Apr 1, 1960·British Heart Journal·M HOLT, S ORAM

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