Document Detail


Familial congenital sinus rhythm anomalies: clinical and pathological correlations.
MedLine Citation:
PMID:  1279540     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe pathological abnormalities in a 72-year-old male member of a family with a congenital absence of sinus rhythm and a tendency to develop atrial fibrillation at an early age, and in a 54-year-old female member of a family with cardiomyopathy and progressive conduction system disease manifested by first-degree atrioventricular (AV) block, left bundle branch block, and atrial arrhythmias. Both patients died suddenly. The absence of sinus rhythm in case 1 could be explained by marked atrophy, degeneration, and isolation of the sinoatrial (SA) node. The SA node was also diseased in the member of the other family with atrial arrhythmias. Additional common features in both cases included: fatty metamorphosis and degenerative changes of the approaches to the SA node, the atrial preferential fibers, and the approaches to the AV node, a small AV node, degenerative changes of the bundle branches, and floppy AV valves. These findings show that the pathological substrate of familial supraventricular arrhythmias consists of a diffuse involvement of the entire conduction system, bearing resemblance to pathological findings in elderly subjects with acquired sick sinus syndrome.
Authors:
S Bharati; B Surawicz; H J Vidaillet; M Lev
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pacing and clinical electrophysiology : PACE     Volume:  15     ISSN:  0147-8389     ISO Abbreviation:  Pacing Clin Electrophysiol     Publication Date:  1992 Nov 
Date Detail:
Created Date:  1992-12-11     Completed Date:  1992-12-11     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7803944     Medline TA:  Pacing Clin Electrophysiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1720-9     Citation Subset:  IM    
Affiliation:
Congenital Heart and Conduction System Center, Christ Hospital and Medical Center Oak Lawn, Illinois.
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MeSH Terms
Descriptor/Qualifier:
Aged
Arrhythmias, Cardiac / congenital*,  genetics*,  pathology
Atrial Fibrillation / genetics,  pathology
Cardiomyopathies / genetics,  pathology
Female
Heart Block / congenital,  pathology
Heart Conduction System / pathology*
Humans
Male
Middle Aged

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


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