Document Detail

The benefits of transplacental treatment of isolated congenital complete heart block associated with maternal anti-Ro/SSA antibodies: a review.
MedLine Citation:
PMID:  20696021     Owner:  NLM     Status:  MEDLINE    
Isolated congenital complete atrio-ventricular block (CAVB) is associated with the transplacental passage of maternal autoantibodies directed to foetal Ro/SSA ribonucleoproteins. Their interactions most likely trigger the inflammation of the atrio-ventricular node and the myocardium in susceptible foetuses. The inflamed tissues may then heal with fibrosis that may cause heart block, endocardial fibroelastosis, and dilated cardiomyopathy. CAVB, the most common cardiac complication, typically develops between 18 and 24 gestational weeks. Untreated, the condition carries a significant mortality risk as the foetus needs to overcome the sudden drop in ventricular rate, the loss of normal atrial systolic contribution to ventricular filling, and perhaps concomitant myocardial inflammation and fibrosis. The rationale to treat a foetus at the stage of CAVB is primarily to mitigate myocardial inflammation and to augment foetal cardiac output. Maternal dexamethasone administration has been shown to improve incomplete foetal AV block, myocardial dysfunction, and cavity effusions. Beta-sympathomimetics may be useful to increase the foetal heart rate and myocardial contractility. Published data from our institution suggest an improved survival >90% if maternal high-dose dexamethasone was initiated at the time of CAVB detection and maintained during the pregnancy and if a beta-adrenergic drug was added at foetal heart rates below 55 beats/min. Despite the improvement in outcome, there is an ongoing debate about treatment-related risks. In this review, we will appraise the natural history of untreated CAVB, discuss currently available management options, and examine the results and risks of in-utero treatment of antibody-mediated CAVB.
D Hutter; E D Silverman; E T Jaeggi
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Scandinavian journal of immunology     Volume:  72     ISSN:  1365-3083     ISO Abbreviation:  Scand. J. Immunol.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-08-10     Completed Date:  2010-10-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0323767     Medline TA:  Scand J Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  235-41     Citation Subset:  IM    
Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada.
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MeSH Terms
Adrenergic beta-Agonists / administration & dosage,  therapeutic use
Antibodies, Antinuclear / immunology*
Atrioventricular Block / congenital*,  etiology,  immunology,  prevention & control,  therapy*
Fetal Diseases / etiology,  immunology,  prevention & control,  therapy
Fetal Therapies / methods*
Maternal-Fetal Exchange / immunology*
Steroids / administration & dosage,  therapeutic use
Reg. No./Substance:
0/Adrenergic beta-Agonists; 0/Antibodies, Antinuclear; 0/SS-A antibodies; 0/Steroids

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