TY - JOUR
T1 - Proposal for a new definition of congenital complete atrioventricular block
AU - Brucato, Antonio
AU - Jonzon, A.
AU - Friedman, D.
AU - Allan, L. D.
AU - Vignati, G.
AU - Gasparini, M.
AU - Stein, J. I.
AU - Montella, S.
AU - Michaelsson, M.
AU - Buyon, J.
PY - 2003
Y1 - 2003
N2 - The classic old definition of congenital heart block by Yater (1929) is still generally accepted: 'Heart block established in a young patient. There must be some evidence of the existence of the slow pulse at a fairly early age and absence of a history of any infection which might cause the condition after birth: notably diphtheria, rheumatic fever, chorea and congenital syphilis'. However, other definitions are used. We systematically reviewed 1825 cases from 38 separate studies. We conclude that complete AV blocks detected in utero in the absence of structural abnormalities differ from blocks detected later in life with respect to pathogenesis (they are generally associated with maternal anti-Ro/SSA antibodies), poorer childhood prognosis, increased risk of developing late-onset dilated cardiomyopathy, different maternal clinical features and increased risk of recurrence in future pregnancies. For these reasons we propose a new modern definition of congenital complete AV block which might be acceptable to cardiologists, rheumatologists, pediatricians and obstetricians: 'an AV block is defined as congenital if it is diagnosed in utero, at birth or within the neonatal period (0-27 days after birth)'.
AB - The classic old definition of congenital heart block by Yater (1929) is still generally accepted: 'Heart block established in a young patient. There must be some evidence of the existence of the slow pulse at a fairly early age and absence of a history of any infection which might cause the condition after birth: notably diphtheria, rheumatic fever, chorea and congenital syphilis'. However, other definitions are used. We systematically reviewed 1825 cases from 38 separate studies. We conclude that complete AV blocks detected in utero in the absence of structural abnormalities differ from blocks detected later in life with respect to pathogenesis (they are generally associated with maternal anti-Ro/SSA antibodies), poorer childhood prognosis, increased risk of developing late-onset dilated cardiomyopathy, different maternal clinical features and increased risk of recurrence in future pregnancies. For these reasons we propose a new modern definition of congenital complete AV block which might be acceptable to cardiologists, rheumatologists, pediatricians and obstetricians: 'an AV block is defined as congenital if it is diagnosed in utero, at birth or within the neonatal period (0-27 days after birth)'.
KW - Congenital atrioventricular block
KW - Fetal heart block
KW - Heart block, congenital (MeSH term)
KW - Neonatal lupus
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U2 - 10.1191/0961203303lu408oa
DO - 10.1191/0961203303lu408oa
M3 - Article
C2 - 12873043
AN - SCOPUS:0038786643
VL - 12
SP - 427
EP - 435
JO - Lupus
JF - Lupus
SN - 0961-2033
IS - 6
ER -