title: The myocardial and coronary histopathology and pathogenesis of hypoplastic left heart syndrome.
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.
authors.affiliation: 1University of Cincinnati,Cincinnati,Ohio,United States of America.
authors.forename: Charles R
authors.initials: CR
authors.lastname: Cole
authors.affiliation: 2Washington University in St. Louis,St. Louis,Missouri,United States of America.
authors.forename: Pirooz
authors.initials: P
authors.lastname: Eghtesady
mesh_terms: D003330:Coronary Vessel Anomalies / Q000150:complications; D003331:Coronary Vessels / Q000473:pathology*; D004695:Endocardial Fibroelastosis / Q000209:etiology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000209:etiology*; D007231:Infant, Newborn; D009206:Myocardium / Q000473:pathology*
keywords: CHD; Hypoplastic left heart syndrome; coronary arteries; endocardial fibroelastosis; myocardial morphology