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The myocardial and coronary histopathology and pathogenesis of hypoplastic left heart syndrome

Published online by Cambridge University Press:  08 July 2015

Charles R. Cole  and
Pirooz Eghtesady
Charles R. Cole
Affiliation:
University of Cincinnati, Cincinnati, Ohio, United States of America
Pirooz Eghtesady*
Affiliation:
Washington University in St. Louis, St. Louis, Missouri, United States of America
*
Correspondence to: P. Eghtesady MD, PhD, Division of Pediatric Cardiothoracic Surgery, Washington University School of Medicine, Campus Box 8234, St. Louis, MO 63110, United States of America. Tel: +314 454 6165; Fax: +314 454 2381; E-mail: eghtesadyp@wudosis.wustl.edu
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Abstract

Hypoplastic left heart syndrome has the greatest mortality rate among all CHDs and without palliation is uniformly fatal. Despite noble efforts, the aetiology of this syndrome is unknown and a cure remains elusive. The genetic and anatomic heterogeneity of hypoplastic left heart syndrome supports a rethinking of old hypotheses and warrants further investigation into the histological and vascular variations recognised with this syndrome. In an effort to elucidate the pathogenesis of hypoplastic left heart syndrome, this review will focus on its unique myocardial and coronary pathology as well as evaluate the association of hypoplastic left heart syndrome with the endocardial fibroelastosis reaction.

Keywords

Hypoplastic left heart syndromeendocardial fibroelastosiscoronary arteriesmyocardial morphologyCHD
Type
Review Articles
Information
Cardiology in the Young , Volume 26 , Issue 1 , January 2016 , pp. 19 - 29
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Copyright
© Cambridge University Press 2015 

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