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Clinicopathological correlation—a case of complex congenitally corrected transposition

Published online by Cambridge University Press:  19 August 2008

Marty Schenk ,
Leon M. Gerlis  and
Jane Somerville
Marty Schenk
Affiliation:
From the Grown-Up Congenital Heart Unit, The Royal Brompton National Heart &Lung Hospital, London
Leon M. Gerlis*
Affiliation:
From the Grown-Up Congenital Heart Unit, The Royal Brompton National Heart &Lung Hospital, London
Jane Somerville
Affiliation:
From the Grown-Up Congenital Heart Unit, The Royal Brompton National Heart &Lung Hospital, London
*
Dr. Leon M. Gerlis, Department of Paediatrics, National Heart & Lung Institute, Dovehouse Street, London SW3 6LY, United Kingdom. Tel, 071 351 8751; Fax. 071 351 8230.
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Summary

A 29-year-old man, with a diagnosis of congenitally corrected transposition (discordant atrioventricular and ventriculoarterial connections) with associated multiple ventricular septal defects, mild pulmonary valvar stenosis and Ebstein's malformation of a stenotic left-sided morphologically tricuspid valve, died after closure of the ventricular septal defect and insertion of a valved homograft conduit between the morphologically left ventricle and the pulmonary trunk. Death occurred because of failure to appreciate the bizarre and rather stenotic anomaly of the morphologically tricuspid valve, in which the anterosuperior leaflet divided the morphologically right ventricle into an inlet portion and a smaller outlet portion. After closure of the ventricular septal defects, this ventricle could no longer support the systemic circulation. We discuss the natural history and the reasons for the erroneous interpretation of the echocardiographic images of the left-sided tricuspid valve.

Keywords

Congenitally corrected transpositionEbstein's malformation of the left-sided atrioventricular valveechocardiogram
Type
Original Articles
Information
Cardiology in the Young , Volume 4 , Issue 3 , July 1994 , pp. 238 - 243
Copyright
Copyright © Cambridge University Press 1994

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References

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