The management of hypoplastic left heart syndrome with a right aortic arch.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Tatum, GH; Morell, VO; Park, SC

Published Date

  • October 2006

Published In

Volume / Issue

  • 16 / 5

Start / End Page

  • 504 - 506

PubMed ID

  • 16984705

International Standard Serial Number (ISSN)

  • 1047-9511

Digital Object Identifier (DOI)

  • 10.1017/S1047951106000448


  • eng

Conference Location

  • England