Document Detail

Hereditary atrial septal defect. Update of a large kindred.
MedLine Citation:
PMID:  148839     Owner:  NLM     Status:  MEDLINE    
A large pedigree wherein members are predisposed to atrial septal defect (ASD) and associated cardiovascular anomalies has been studied. There were ten direct line relatives with ASD verified by cardiac catheterization and/or surgery and in one case by reliable medical history. The pedigree shows vertical transmission of ASD through four generations. Estimates of the genetic segregation ratio were near 50%, indicating that predisposition to ASD among family members was due to a deleterious autosomal dominant gene. Family members have also manifested other congenital heart anomalies, consistent with pleiotropic effects of the putative gene. Electrocardiograms in six ASD patients revealed an absence of prolonged P-R interval. In the light of prior studies showing prolonged P-R interval in familial ASD, we suggest the existence of at least two distinct hereditary varieties of ASD, one with and one without a prolonged P-R interval.
H T Lynch; K Bachenberg; R E Harris; W Becker
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of diseases of children (1960)     Volume:  132     ISSN:  0002-922X     ISO Abbreviation:  Am. J. Dis. Child.     Publication Date:  1978 Jun 
Date Detail:
Created Date:  1978-07-26     Completed Date:  1978-07-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0370471     Medline TA:  Am J Dis Child     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  600-4     Citation Subset:  AIM; IM    
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MeSH Terms
Cardiomegaly / complications
Genes, Dominant
Heart Block / complications
Heart Defects, Congenital / complications
Heart Murmurs
Heart Septal Defects, Atrial / complications,  genetics*
Infant, Newborn
Middle Aged
Tachycardia / complications
Wolff-Parkinson-White Syndrome / complications,  genetics

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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