Non-compaction cardiomyopathy: a genetically and clinically heterogeneous disorder:  Presentation of two cases and review of the literature

I. Rooms; K. Dujardin; J. De Sutter

Document Details :

Title: Non-compaction cardiomyopathy: a genetically and clinically heterogeneous disorder
Subtitle: Presentation of two cases and review of the literature
Author(s): I. Rooms , K. Dujardin , J. De Sutter
Journal: Acta Cardiologica
Volume: 70 Issue: 6 Date: 2015
Pages: 625-631
DOI: 10.2143/AC.70.6.3120173

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.