title: Isolated left ventricular non-compaction: the case for abnormal myocardial development.
abstract: Isolated ventricular non-compaction is an increasingly commonly diagnosed myocardial disorder characterised by excessive and prominent trabeculation of the morphologically left, and occasionally the right, ventricle. This is associated with high rates of thromboembolism, cardiac failure, and cardiac arrhythmia. Recent improvements in understanding the embryonic processes underlying ventricular formation have led to the hypothesis that ventricular non-compaction is due to a failure of normal ventriculogenesis, leading to abnormal myocardium which may present clinically many years later. Experimental work in animal models provides several candidate transcription factors and signalling molecules that could, in theory, cause ventricular non-compaction if disrupted.
authors.affiliation: Department of Clinical Pharmacology, BHF Laboratories, University College, London. rbrecke@nimr.mrc.ac.uk
authors.forename: Ross A
authors.initials: RA
authors.lastname: Breckenridge
authors.forename: Robert H
authors.initials: RH
authors.lastname: Anderson
authors.forename: Perry M
authors.initials: PM
authors.lastname: Elliott
mesh_terms: D000217:Acyltransferases; D000818:Animals; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism; D004452:Echocardiography; D020022:Genetic Predisposition to Disease; D006321:Heart / Q000196:embryology; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism; D006352:Heart Ventricles / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006801:Humans; D009154:Mutation; D009206:Myocardium / Q000473:pathology; D010641:Phenotype; D011506:Proteins / Q000235:genetics / Q000378:metabolism; D012307:Risk Factors; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism
chemical_list: D016189:Dystrophin; D011506:Proteins; D014157:Transcription Factors; D000217:Acyltransferases; C108675:TAFAZZIN protein, human