Non-compaction of the ventricular myocardium （NVM） is a rare congenital genetic heart defect that was initially reported 17 years ago by means of autopsy; few cases have been published since then. It has been classi...Non-compaction of the ventricular myocardium （NVM） is a rare congenital genetic heart defect that was initially reported 17 years ago by means of autopsy; few cases have been published since then. It has been classified as the primary inherited cardiomyopathy by the American Heart Association Scientific Statement in 2006 under contemporary definitions and classification of cardiomyopathies. It is caused by an arrested development of muscle fiber compaction, a process that normally takes place dunng early embryogenesls, which is characterized by numerous sinusoids or trabeculae that are excessive in number and abnormal in prominence and by deep intratrabecular recesses covered by endothelium that exhibits continuity with ventricular endocardium. It usually involves one and/or more segments of the left ventricle, while the clinical manifestations are highly variable,展开更多
文摘Non-compaction of the ventricular myocardium （NVM） is a rare congenital genetic heart defect that was initially reported 17 years ago by means of autopsy; few cases have been published since then. It has been classified as the primary inherited cardiomyopathy by the American Heart Association Scientific Statement in 2006 under contemporary definitions and classification of cardiomyopathies. It is caused by an arrested development of muscle fiber compaction, a process that normally takes place dunng early embryogenesls, which is characterized by numerous sinusoids or trabeculae that are excessive in number and abnormal in prominence and by deep intratrabecular recesses covered by endothelium that exhibits continuity with ventricular endocardium. It usually involves one and/or more segments of the left ventricle, while the clinical manifestations are highly variable,
