Document Detail


Pathogenesis of cardiac conduction disorders in children genetic and histopathologic aspects.
MedLine Citation:
PMID:  16969859     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fetal dysrhythmias are usually transient. Abnormal fetal rates and rhythms during labor are "functional." Fetal dysrhythmias may be associated with congenital heart disease and fetal hydrops. Bradycardia is usually related to fetal distress; supraventricular tachycardia, atrial flutter, and atrial fibrillation may be associated with severe congestive heart failure. Ventricular fibrillation is rare in the fetus and infant and is usually associated with myocardial necrosis with perimembranous septal defect; the nonbranching atrioventricular (AV) bundle may have an aberrant position and result in cardiac arrhythmia. Wolff-Parkinson-White syndrome with conduction abnormalities and left ventricular hypertrophy (LVH) is due to an accessory pathway that bypasses the AV sulcus and results in faster conduction. Carnitine deficiency may be primary or secondary and may result in cardiac arrhythmia. Histiocytoid cardiomyopathy is characterized by cardiomegaly, incessant ventricular tachycardia, and frequently sudden death. Arrhythmogenic right ventricular dysplasia (ARVD) results in ventricular tachycardia and left bundle branch block. Noncompaction of the left ventricle predisposes to potentially fatal arrhythmias. Long Q-T syndromes (LQTS) are a heterogeneous group of disorders with many genetic mutations. Brugada syndrome is an autosomal dominant trait with right bundle branch block and ST elevation. Barth syndrome is an X-linked disorder with dilated cardiomyopathy, cyclic neutropenia and skeletal myopathy. Hypertrophic cardiomyopathy in infancy may be related to metabolic diseases, particularly glycogen storage diseases; the familial form predisposes to sudden death. Arrhythmias following cardiac surgery may occur after closure of a ventricular septal defect (VSD) or damage to the conduction system.
Authors:
Enid Gilbert-Barness; Lewis A Barness
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  140     ISSN:  1552-4825     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2006 Oct 
Date Detail:
Created Date:  2006-09-28     Completed Date:  2006-11-29     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1993-2006     Citation Subset:  IM    
Affiliation:
Department of Pathology, University of South Florida College of Medicine, Tampa General Hospital, Tampa, Florida 33606, USA. egilbert@tgh.org
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MeSH Terms
Descriptor/Qualifier:
Arrhythmias, Cardiac / etiology*,  genetics,  pathology,  physiopathology
Cardiomyopathy, Hypertrophic / genetics
Carnitine / deficiency
Female
Heart Block / congenital,  genetics
Heart Conduction System / pathology,  physiopathology
Humans
Infant
Infant, Newborn
Long QT Syndrome / genetics
Pregnancy
Syndrome
Chemical
Reg. No./Substance:
541-15-1/Carnitine

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


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