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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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References

Chen MR, Chiu IS, Chen BF. Hypoplastic left heart syndrome with right aortic arch in a newborn. Br Heart J 1993; 69: 449–450.Google Scholar
Patel CR, Spector ML, Zahka KG. Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery. Cardiol Young 1999; 9: 331–334.Google Scholar
Elzenga NJ, Gittenberger-de Groot AC. Coarctation and related aortic arch anomalies in hypoplastic left heart syndrome. Int J Cardiol 1985; 8: 379–389.Google Scholar
Devloo-Blancquaert A, Titus JL, Edwards JE, Vallaeys JHJ, De Gezelle HRM, Coppens M. Interruption of aortic arch and hypoplastic left heart syndrome. Pediatr Cardiol 1995; 16: 304–308.Google Scholar
Knight L, Edwards JE. Right aortic arch: types and associated cardiac anomalies. Circulation 1974; 50: 1047–1051.Google Scholar
Hastreiter AR, D'Cruz IA, Cantez T. Right-sided aorta: part I: Occurrence of right aortic arch in various types of congenital heart disease. Br Heart J 1966; 28: 722–725.Google Scholar
D'Cruz IA, Cantez T, Namin EP, Licata R, Hastreiter AR. Right-sided aorta: part II: Right aortic arch, right descending aorta and associated anomalies. Br Heart J 1966; 28: 725–739.Google Scholar
Park SC, Zuberbuhler JR. Vascular ring and pulmonary sling. In: Anderson RH, Baker EJ, MacArtney FJ, Rigby ML, Shinebourne EA, Tynan M (eds). Pediatric Cardiology, 2nd Ed. Churchill-Livingstone, London, UK, 2002, pp 1559–1575.