| Familial congenital sinus rhythm anomalies: clinical and pathological correlations. | |
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MedLine Citation:
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PMID: 1279540 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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S Bharati; B Surawicz; H J Vidaillet; M Lev |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Pacing and clinical electrophysiology : PACE Volume: 15 ISSN: 0147-8389 ISO Abbreviation: Pacing Clin Electrophysiol Publication Date: 1992 Nov |
Date Detail:
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Created Date: 1992-12-11 Completed Date: 1992-12-11 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7803944 Medline TA: Pacing Clin Electrophysiol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1720-9 Citation Subset: IM |
Affiliation:
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Congenital Heart and Conduction System Center, Christ Hospital and Medical Center Oak Lawn, Illinois. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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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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