title: Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases?
abstract: Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend different strategies for diagnosing and treating patients with LVNC. This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinical manifestations, treatment modalities, anatomy and pathology, embryology, genetics, epidemiology, and imaging. Three markers currently define LVNC: prominent left ventricular trabeculae, deep intertrabecular recesses, and a thin compacted layer. Although new genetic data from mice and humans supports LVNC as a distinct cardiomyopathy, evidence for LVNC as a shared morphological trait is not ruled out. Criteria supporting LVNC as a shared morphological trait may depend on consensus guidelines from the multiple professional organizations. Enhanced imaging and increased use of genetics are both predicted to significantly impact our overall understanding of the basic mechanisms causing LVNC and its optimal management.
authors.affiliation: Centre for Inherited Cardiovascular Disease, IRCCS Foundation Policlinico San Matteo, Pavia, Italy.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Arbustini
authors.affiliation: Department of Internal Medicine, University Hospital Würzburg, and Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Weidemann
authors.affiliation: Lillehei Heart Institute and Division of Cardiology, Department of Medicine, University of Minnesota, Minneapolis, Minnesota. Electronic address: JLHALL@umn.edu.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Hall
mesh_terms: D000818:Animals; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000503:physiopathology; D004452:Echocardiography; D017709:Fractals; D006331:Heart Diseases / Q000175:diagnosis* / Q000503:physiopathology; D006352:Heart Ventricles / Q000503:physiopathology*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D009682:Magnetic Resonance Spectroscopy; D051379:Mice; D009154:Mutation; D010641:Phenotype; D015398:Signal Transduction; D013927:Thrombosis / Q000503:physiopathology; D016896:Treatment Outcome; D018608:Ultrasonography, Doppler
keywords: compacted; epidemiology; genetics; imaging; pathology; trabeculae