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Proposal for a new definition of congenital complete atrioventricular block

Divisione Medica ‘Brera’ e Reumatologia, Ospedale Niguarda Ca’ Granda, Milan, Italy, abrucato{at}ospedaleniguarda.it

A Jonzon

Children’s Hospital, Uppsala University, Uppsala, Sweden

D Friedman

St. Luke’s - Roosevelt Hospital Center, New York

L D Allan

Babies’ and Children’s Hospital, Columbia Presbyterian Medical Centre, New York, USA

G Vignati

Cardiologia Pediatrica, Ospedale Niguarda Ca’ Granda, Milan, Italy

M Gasparini

U.O. Elettrofisiologia-Elettrostimolazione, Istituto Clinico Humanitas, Rozzano, Milan, Italy

J I Stein

Abteilung fur Pediatrische Kardiologie, Universitatsklinik fur Kinder und Jugendheilkunde, Graz, Austria

S Montella

Servizio di Cardiologia, Ospedale dei Bambini ‘V. Buzzi’, Milan, Italy

M Michaelsson

Children’s Hospital, Uppsala University, Uppsala, Sweden

J Buyon

Hospital for Joint Diseases, New York University School of Medicine, New York

The classic old definition of congenital heart block by Yater (1929) is still generally accepted: ‘Heart block establishedin a young patient. There must be some evidenceof the existenceof 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 systematicallyreviewed 1825 cases from 38 separatestudies. 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 congenitalcomplete 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)’.

Key Words: congenitalatrioventricularblock • fetal heart block • heart block, congenital(MeSH term) • neonatal lupus

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