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Congenital absence of aortic valvar leaflets: a rare variant of the hypoplastic left heart syndrome

Published online by Cambridge University Press:  15 August 2006

Kenneth P. Rouillard ,
Phillip Moore  and
Norman H. Silverman
Kenneth P. Rouillard
Affiliation:
Division of Pediatric Cardiology, The University of California, San Francisco, San Francisco, California, USA
Phillip Moore
Affiliation:
Division of Pediatric Cardiology, The University of California, San Francisco, San Francisco, California, USA
Norman H. Silverman
Affiliation:
Division of Pediatric Cardiology, The University of California, San Francisco, San Francisco, California, USA
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Abstract

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Congenital absence of aortic valvar leaflets is a rare and fatal variant of the hypoplastic left heart syndrome. We describe a recent patient seen at our institution with this lesion, illustrating a combined echocardiographic and angiographic approach that delineates both anatomy and physiology. The early mortality experienced in previous reports, as well as unsuccessful surgical palliation in our case, should promote further discussion regarding the optimal treatment.

Keywords

Aortic valvar leaflet aplasiaaortic insufficiencyhypoplastic left heart syndrome
Type
Brief Report
Information
Cardiology in the Young , Volume 11 , Issue 4 , July 2001 , pp. 453 - 457
Copyright
2001 Cambridge University Press