| Left ventricular hypoplasia a spectrum of disease involving the left ventricular outflow tract, aortic valve, and aorta. | |
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MedLine Citation:
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PMID: 22192721 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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"Hypoplastic left heart syndrome" is an unsatisfactory term describing lethal underdevelopment of the left ventricle (LV). It represents the more severe end of a spectrum of LV hypoplasia, mandating single-ventricle palliation or cardiac transplantation. Less severe "borderline" ventricular hypoplasia may instead allow various biventricular therapeutic strategies and better long-term outcomes. In this review, we consider factors causing and modifying the abnormal development of the LV. LV hypoplasia is typically seen in association with left ventricular outflow tract obstruction, itself part of a spectrum of related defects with common etiologies. Secondary responses to outflow obstruction are complex but involve abnormal flow dynamics and shear stresses that result in compromised and poorly orchestrated ventricular growth and development. Subsequent remodeling is likely influenced by genetic modifiers, including intrinsic myocardial growth signaling pathways, possibly including those of HAND transcription factors. In addition, during the latter stages of gestation, cardiomyocytes undergo a switch in myogenic potential and lose the ability to undergo mitosis. Ventricular hyperplasia can therefore no longer occur; remodeling is instead limited to muscular hypertrophy. Subtle differences in this switch in myogenic potential-and modulators thereof-are likely to be of clinical and therapeutic importance, especially in children with "borderline LVs" being considered for fetal interventions or post-natal biventricular repair strategies. Finally, by more clearly understanding the initiators and propagators of abnormal ventricular development, we can hope to lean away from grouping a heterogeneous group of infants together under the unsatisfactory term "hypoplastic left heart syndrome." |
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Authors:
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Edward J Hickey; Christopher A Caldarone; Brian W McCrindle |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of the American College of Cardiology Volume: 59 ISSN: 1558-3597 ISO Abbreviation: J. Am. Coll. Cardiol. Publication Date: 2012 Jan |
Date Detail:
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Created Date: 2011-12-23 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8301365 Medline TA: J Am Coll Cardiol Country: United States |
Other Details:
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Languages: eng Pagination: S43-54 Citation Subset: AIM; IM |
Copyright Information:
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Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. |
Affiliation:
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Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, the Hospital for Sick Children, Toronto, Ontario, Canada. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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