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.
Bibliographical noteFunding Information:
Dr. Arbustini was supported by the European Union INHERITANCE project #241924 and Italian Ministry of Health “Diagnosis and Treatment of Hypertrophic Cardiomyopathies” (#RF-OSM-2008-1145809) IRCCS Policlinico San Matteo, Pavia. Dr. Hall was supported by the John and Nancy Lindahl Foundation and National Heart, Lung, and Blood Institute grant 1R21DK078029-01 ; and serves as a consultant for USF Health. Dr. Weidemann has reported that he has no relationships relevant to the contents of this paper to disclose.
© 2014 American College of Cardiology Foundation.