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a Paediatrics,
National Heart and Lung Institute, Royal Brompton Campus, Imperial
College of Science, Technology and Medicine, Dovehouse Street, London
SW3 6LY, UK, b Fetal and Perinatal
Cardiology, Guy's Hospital, London SE1, UK, c Department of Anatomy, Institute of Child Health
at Alder Hey, Liverpool, UK
Correspondence to: Dr Ho email: yen.ho{at}ic.ac.uk
Accepted 23 November
1999
OBJECTIVE
To review the anatomical structure of the right
atrioventricular junction, including the specialised atrioventricular
conduction system, in hearts with Ebstein's malformation, to identify
potential substrates for the abnormalities in conduction.
METHODSFive heart specimens representing the
morphological spectrum of Ebstein malformation were examined grossly
and histologically.
RESULTSOn the endocardial surface, the atrioventricular
junction was marked by a faint line in two hearts, and by a small ridge
in the other three. Analysis of the right parietal junction in four hearts revealed only two accessory muscular atrioventricular
connections. A plane of fibrofatty tissue separated atrial from
ventricular myocardium in the right parietal junction in all hearts.
The compact atrioventricular node was closer to the coronary sinus than
usual. Accessory nodoventricular connections were present in four
hearts, while accessory fasciculo-ventricular connections were found in one. The right bundle branch was hypoplastic or absent in four hearts.
CONCLUSIONSIn this small series, the parietal
atrioventricular junction was better developed than previously thought.
Structural abnormalities of the atrioventricular conduction system,
however, were present. These may account for some of the conduction
abnormalities frequently observed with the Ebstein malformation.
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