title: Non-compaction cardiomyopathy: a genetically and clinically heterogeneous disorder.
abstract: Left ventricular non-compaction (LVNC) is a rare disease that can occur isolated or in association with other disorders, including congenital heart disease and musculoskeletal disorders. It is characterized by a two-layered myocardium with excessive trabeculation of the left ventricle. Diagnosis is challenging as left ventricular trabeculations can be pathological yet can also be a normal finding in athletes and black people, leading to overdiagnosis. Echocardiography and CMR are important diagnostic tools. LVNC is often complicated by ventricular dysfunction, arrhythmias and thromboembolic events. Based on two cases, we review the pathogenesis, genetic background, clinical features and treatment of LVNC according to the available guidelines.
authors.forename: Isabel
authors.initials: I
authors.lastname: Rooms
authors.forename: Karl
authors.initials: K
authors.lastname: Dujardin
authors.forename: Johan
authors.initials: J
authors.lastname: De Sutter
mesh_terms: D000293:Adolescent; D000368:Aged; D003937:Diagnosis, Differential; D004452:Echocardiography; D005260:Female; D005820:Genetic Testing; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D019028:Magnetic Resonance Imaging, Cine
keywords: Left ventricular non-compaction; cardiomyopathy; echocardiography; genetics; heart failure