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Surgical treatment of Ebstein's malformation: state of the art in 2006

Published online by Cambridge University Press:  13 October 2006

Joseph A. Dearani ,
Patrick W. O'Leary  and
Gordon K. Danielson
Joseph A. Dearani
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, Minnesota, United States of America
Patrick W. O'Leary
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic and Foundation, Rochester, Minnesota, United States of America
Gordon K. Danielson
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, Minnesota, United States of America
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Abstract

Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet (Fig. 1). The second feature is anterior and apical rotational displacement of the functional annulus (Fig. 2). The third abnormality is dilation of the “atrialized” portion of the right ventricle, with variable degrees of hypertrophy and thinning of the wall. The fourth finding is redundancy, fenestrations, and tethering of the anterior leaflet. A fifth abnormality is dilation of the right atrioventricular junction, this being the true tricuspid valvar annulus. The final feature is variable ventricular myocardial dysfunction. Each heart with Ebstein's malformation is different, and there is an infinite variability that can occur with the above mentioned characteristics. These anatomical and functional abnormalities cause important tricuspid regurgitation, which results in right atrial and right ventricular dilation, and atrial and ventricular arrhythmias.

Keywords

Ebstein's anomalycongenital heart defectstricuspid valve
Type
The Atrioventricular Valves
Information
Cardiology in the Young , Volume 16 , Supplement S3: Controversies and Challenges of the Atrioventricular Junctions and Other Challenges Facing Paediatric Cardiovascular Practitioners and their Patients , September 2006 , pp. 12 - 20
Copyright
© 2006 Cambridge University Press

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