| Impact of noncardiac congenital and genetic abnormalities on outcomes in hypoplastic left heart syndrome. | |
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MedLine Citation:
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PMID: 20494032 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Hypoplastic left heart syndrome may coexist with noncardiac congenital defects or genetic syndromes. We explored the impact of such lesions on outcomes after staged univentricular palliation. METHODS: Society of Thoracic Surgeons database 2002 to 2006: Children diagnosed with hypoplastic left heart syndrome who underwent stage 1 Norwood (n = 1,236), stage 2 superior cavopulmonary anastamosis (n = 702) or stage 3 Fontan (n = 553) procedures were studied. In-hospital mortality, postoperative complications, and length of stay were compared at each stage between those with and without noncardiac-genetic defects. Congenital Heart Surgeons' Society database 1994 to 2001: All 703 infants enrolled in the Congenital Heart Surgeons' Society critical left ventricular outflow tract obstruction study who underwent primary stage 1 palliation were reviewed. The impact of noncardiac defects-syndromes on survival was explored using multivariable parametric models with bootstrap bagging. RESULTS: Society of Thoracic Surgeons database: Stage 1 in-hospital mortality (26% vs 20%, p = 0.04) and mean postoperative length of stay (42 versus 31 days, p < 0.0001) were greater, and postoperative complications significantly more prevalent in infants with noncardiac-genetic defects. Congenital Heart Surgeons' Society database: Noncardiac-genetic defects were present in 55 (8%). Early hazard for death after Norwood was significantly worse in infants with noncardiac defects-syndromes (p = 0.008). Chromosomal defects (n = 14) were highly unfavorable: the early risk of death was doubled (10-year survival 25 +/- 9% vs 54 +/- 2%, p = 0.005). Turner syndrome accounted for the majority of chromosomal defects in this population (11 of 14, 79%). Mode of death was rarely attributable to the noncardiac-genetic defect. CONCLUSIONS: Survival in hypoplastic left heart syndrome is strongly influenced by the presence of noncardiac abnormalities. Strategies to improve mortality in infants with noncardiac abnormalities should be explored. Presence of chromosomal defects, especially Turner syndrome, should enter decision-management options for parents and physicians. |
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Authors:
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Angira Patel; Edward Hickey; Constantine Mavroudis; Jeffrey P Jacobs; Marshall L Jacobs; Carl L Backer; Melanie Gevitz; Constantine D Mavroudis |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The Annals of thoracic surgery Volume: 89 ISSN: 1552-6259 ISO Abbreviation: Ann. Thorac. Surg. Publication Date: 2010 Jun |
Date Detail:
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Created Date: 2010-05-24 Completed Date: 2010-06-16 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 15030100R Medline TA: Ann Thorac Surg Country: Netherlands |
Other Details:
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Languages: eng Pagination: 1805-13; discussion 1813-4 Citation Subset: AIM; IM |
Copyright Information:
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2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved. |
Affiliation:
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Division of Cardiology, Children's Memorial Hospital, Northwestern University, Chicago IL, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cardiac Surgical Procedures
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methods Congenital Abnormalities* Genetic Diseases, Inborn / complications* Humans Hypoplastic Left Heart Syndrome / complications*, surgery* Infant Risk Factors Treatment Outcome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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