title: Isolated left ventricular noncompaction: what do we really know?
abstract: Isolated left ventricular noncompaction (ILVNC) is a myocardial disorder thought to occur as a result of an arrest in embryogenesis. Recently, it has been classified as a genetic cardiomyopathy. Familial and sporadic forms of ILVNC have been documented. There is considerable genetic heterogeneity among familial forms, although sarcomeric mutations are the most common genetic mutations. Issues related to familial screening require careful consideration and future research. There is considerable controversy regarding several aspects relating to pathogenesis, diagnosis, and management of subjects with ILVNC. Differentiating some cases of ILVNC from normal variant myocardial architecture is challenging and requires future work. This review is intended to provide clinicians insight into several aspects of these challenging issues and offer some practical guidance in dealing with some of these difficult clinical scenarios.
authors.affiliation: Department of Cardiology, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Bertsham 2013, Johannesburg, South Africa. ferande.peters@gmail.com
authors.forename: Ferande
authors.initials: F
authors.lastname: Peters
authors.forename: Bijoy K
authors.initials: BK
authors.lastname: Khandheria
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000175:diagnosis* / Q000196:embryology / Q000235:genetics / Q000628:therapy; D008279:Magnetic Resonance Imaging / Q000379:methods; D009154:Mutation; D011379:Prognosis; D013318:Stroke Volume / Q000502:physiology