Document Detail


Apoptosis as a possible cause of gradual development of complete heart block and fatal arrhythmias associated with absence of the AV node, sinus node, and internodal pathways.
MedLine Citation:
PMID:  8641033     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Gradually progressive development of complete heart block in young people often is associated with cardiac arrhythmia and sudden death, but the pathogenesis remains unexplained. METHODS AND RESULTS: A young woman with complete heart block died suddenly. Her mother had serological but no clinical evidence of antiphospholipid syndrome. Five brothers of another family had arrhythmia and heart block. Three died suddenly; the other two have automatic defibrillators and are alive. The hearts from the young woman and two of the three brothers who died were available for our histological examination of their cardiac conduction systems. In two of the three hearts, the AV node was absent; in the third heart, only fragments of the AV node remained. In all three hearts, the sinus node was nearly destroyed by a noninflammatory degeneration with no abnormal fibrosis or infiltrate. In each heart, the interatrial and internodal pathways were similarly involved, and in the young woman, there were no myocardial cells in which these pathways normally exist. CONCLUSIONS: In these three subjects with progressive development of complete heart block and various arrhythmias, all of whom died suddenly, the histological abnormalities of their cardiac conduction systems are best interpreted as resulting from apoptosis. Programmed cell death is a logical explanation for the pathogenesis of this puzzling clinical picture.
Authors:
T N James; E St Martin; P W Willis; T O Lohr
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Circulation     Volume:  93     ISSN:  0009-7322     ISO Abbreviation:  Circulation     Publication Date:  1996 Apr 
Date Detail:
Created Date:  1996-07-17     Completed Date:  1996-07-17     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1424-38     Citation Subset:  AIM; IM    
Affiliation:
Department of Medicine, World Health Organization Cardiovascular Center, University of Texas Medical Branch, Galveston 77555-0129, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adrenergic beta-Antagonists / therapeutic use
Adult
Apoptosis*
Arrhythmias, Cardiac / etiology*,  pathology
Atrioventricular Node / pathology*
Bradycardia / etiology
Cardiac Pacing, Artificial
Child
Death, Sudden, Cardiac / etiology*,  pathology
Defibrillators, Implantable
Disease Progression
Fatal Outcome
Female
Heart Block / complications*,  etiology*,  genetics,  pathology,  therapy
Heart Conduction System / pathology*
Heart Defects, Congenital / complications
Humans
Male
Myocardium / pathology*
Sinoatrial Node / pathology*
Syncope / etiology
Chemical
Reg. No./Substance:
0/Adrenergic beta-Antagonists
Comments/Corrections
Comment In:
Circulation. 1997 Oct 21;96(8):2735   [PMID:  9355920 ]

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


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