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The management of hypoplastic left heart syndrome with aright aortic arch

Published online by Cambridge University Press:  20 September 2006

Gregory H. Tatum ,
Victor O. Morell  and
Sang C. Park
Gregory H. Tatum
Affiliation:
Division of Cardiology, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America
Victor O. Morell
Affiliation:
Division of Cardiothoracic Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America
Sang C. Park
Affiliation:
Division of Cardiology, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America
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Abstract

The combination of hypoplastic left heart syndrome and a right-sided aortic arch is extremely rare and lethal. To the best of our knowledge, no patient with this combination has previously been reported as surviving initial palliation. The anatomic variant is associated with abnormalities in the arteries branching from the aortic arch, making it difficult to construct a reliable source of flow of blood to the lungs. We present here a patient with this combination who survived an initial Damus-Kay-Stansel procedure combined with placement of a conduit from the right ventricle to the pulmonary arteries, and who has subsequently undergone a successful bidirectional cavopulmonary anastomosis. We believe that the conduit placed from the right ventricle provides the most reliable source of flow of blood to the lungs at the time of initial palliation in this usual combination of cardiac lesions.

Keywords

Hypoplasia of the left heartaberrant left subclavian arterydiverticulum of KommerellSano procedure
Type
Brief Report
Information
Cardiology in the Young , Volume 16 , Issue 5 , October 2006 , pp. 504 - 506
Copyright
© 2006 Cambridge University Press

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