作者: Eloisa Arbustini , Frank Weidemann , Jennifer L. Hall
DOI: 10.1016/J.JACC.2014.08.030
关键词: Genetic data 、 Internal medicine 、 Cardiology 、 Trait 、 Left ventricular noncompaction 、 Medicine 、 Cardiomyopathy 、 Genetic counseling 、 Optimal management 、 Morphological trait 、 Genetic testing
摘要: Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend 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 pathology, embryology, genetics, epidemiology, imaging. Three markers currently define LVNC: prominent trabeculae, deep intertrabecular recesses, thin compacted layer. Although genetic data mice humans supports LVNC as cardiomyopathy, evidence morphological not ruled out. Criteria supporting may depend on consensus multiple organizations. Enhanced imaging increased use of genetics are both predicted significantly impact our overall understanding causing optimal management.