?url_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Adc&rft.title=Do+All+Children+with+Congenital+Complete+Atrioventricular+Block+Require+Permanent+Pacing+%3F%0A&rft.creator=Balmer%2C+Christian&rft.creator=Bauersfeld%2C+Urs&rft.subject=Indian+Pacing+and+Electrophysiology+Journal&rft.description=With+an+incidence+of+1+in+20%E2%80%99000+live+born+infants1%2C+congenital+complete+atrioventricular+block+(CCAVB)+is+a+rare+disease.+The+aetiology+is+not+completely+understood.+However%2C+CCAVB+may+be+isolated+or+combined+with+congenital+heart+diseases+in+up+to+53%25+of+affected+individuals2.+Isolated+CCAVB+is+in+up+to+98%25+of+the+children+associated+with+positive+autoimmune+antibodies+in+the+maternal+serum+(anti-Ro%2FSS-A+and+anti-LA%2FSS-B)3%2C4.+Interestingly%2C+these+antibodies+are+not+specifically+directed+against+the+conduction+system+but+also+against+normal+myocardial+cells+and+may+cause+myocarditis5%2C6.+Affection+of+the+conduction+system+can+occur+at+different+levels7.+Histologically%2C+the+atrioventricular+node+tissue+may+be+replaced+by+fibrous+fatty+tissue+with+variable+involvement+of+the+distal+conduction+system8.+%0A++++++++++++The+onset+of+clinical+symptoms+in+patients+with+CCAVB+is+already+antenatally+in+up+to+28%259%2C+but+can+also+occur+only+later+in+life.+This+is+due+to+a+variable+degree+of+heart+block+and+heart+rate.+Most+of+the+symptoms+are+related+to+the+slow+heart+rate%3A+hydrops+foetalis%2C+heart+failure+of+the+neonate%2C+exercise+intolerance+of+the+child.+Longer+pauses+may+cause+praesyncope%2C+syncope+(classical+Adams+Stokes+attacks)+or+even+sudden+cardiac+death.+Whether+or+not+Cardiomegaly+is+mainly+the+result+of+a+chronic+compensatory+increased+stroke+volume+secondary+to+the+slow+heart+rate+is+somewhat+controversial10.+Cardiomegaly+may+also+be+a+distinct+disease+in+a+subgroup+of+patients+because+it+does+not+necessarily+resolve+with+pacemaker+(PM)+therapy11%2C12.+Morbidity+and+mortality+of+CCAVB+do+not+seem+to+correlate+with+antibody+status+or+associated+cardiac+lesions13%2C14.+There+are+case+reports%2C+that+fetal+CCAVB+can+be+improved+with+steroids15.+Rarely%2C+CCAVB+resolves+spontaneously16%2C17%2C18%2C19.+In+most+patients%2C+the+degree+of+conduction+abnormality+will+either+persist+or+worsen+over+time.%0A&rft.publisher=Indian+Pacing+and+Electrophysiology+Group&rft.contributor=Singh%2C+Balbir&rft.contributor=Lokhandwala%2C+Yash&rft.contributor=Francis%2C+Johnson&rft.contributor=Gupta%2C+Anup&rft.date=2003-07&rft.type=Journal+(Paginated)&rft.type=PeerReviewed&rft.format=text%2Fhtml&rft.identifier=http%3A%2F%2Fcogprints.org%2F4281%2F1%2Fbalmer.htm&rft.identifier=++Balmer%2C+Christian+and+Bauersfeld%2C+Urs++(2003)+Do+All+Children+with+Congenital+Complete+Atrioventricular+Block+Require+Permanent+Pacing+%3F++%5BJournal+(Paginated)%5D+++++&rft.relation=http%3A%2F%2Fcogprints.org%2F4281%2F