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Pathophysiology, clinical course and management of congenital complete atrioventricular block.
MedLine Citation:
PMID:  23276818     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
The incidence of congenital atrioventricular (AV) block is between 1 in 15000 and 1 in 20000 births. It may occur in isolation or as a consequence of anomalous development of the conduction tissue in the context of a cardiac malformation. In this review, we use the term "congenital AV bock" to describe complete heart block when it is diagnosed in utero or at birth. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of trans-placental passage of maternal anti-SSA/Ro-SSB/La antibodies. Many mothers are asymptomatic carriers, less than ⅓ have a preexisting diagnosis of a rheumatological disorder. AV block that develops months or years after birth probably occurs as a result of a different disease process that is poorly understood. The diagnosis of congenital AV block is usually confirmed by fetal echocardiography before birth, and by electrocardiography after birth. The implantation of a pacemaker is recommended for symptomatic patients and for asymptomatic patients presenting with profound bradycardia, left ventricular dysfunction, a wide QRS or a prolonged QT interval. It is now recognized that a subset of paced patients develop dilated cardiomyopathy and heart failure, and therefore regular follow-up is important. They are also at high risk of developing complications related to the presence of intracardiac material as a result of prolonged exposure to pacing materials. Future areas of research to minimize these risks include assessing the impact of alternative stimulation sites and the development of new cardiac stimulation techniques.
Authors:
Pierre Bordachar; Zachary Whinnett; Sylvain Ploux; Louis Labrousse; Michel Haissaguerre; Jean-Benoit Thambo
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-12-28
Journal Detail:
Title:  Heart rhythm : the official journal of the Heart Rhythm Society     Volume:  -     ISSN:  1556-3871     ISO Abbreviation:  Heart Rhythm     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2013-1-1     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101200317     Medline TA:  Heart Rhythm     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012. Published by Elsevier Inc.
Affiliation:
University Bordeaux 2, 33000 Bordeaux France; University Hospital of Bordeaux, 33000 Bordeaux, France. Electronic address: bordacharp@hotmail.com.
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