Non-compaction of left ventricle

Non-compaction of left ventricle Non-compaction of left ventricle is often classified as a cardiomyopathy and is characterized by abnormal deep trabeculations, which is more at the apex [1]. Left ventricular non-compaction can be associated with left ventricular dilatation or hypertrophy. Systolic and diastolic dysfunction can occur and other congenital heart diseases can be associated. Ventricular arrhythmias and complete atrioventricular block can occur and present as syncope or sudden cardiac death. Genetic transmission has been noted in 30-50% of patients and several genes have been implicated. NOTCH signaling pathway seems to be the final common pathway affected [1]. It was thought that embryological arrest of normal endomyocardial morphogenesis was the cause of non-compaction. But some authors have disputed this theory [2]. In the early stages of development of the heart when the coronary circulation is yet to develop, thin trabeculae prevent ischemia by providing good surface area for direct absorption from the blood in the cavity. Initially trabecular growth is rapid and accounts for most of the ventricular mass. Later on the trabecular growth becomes slower compared to the compact ventricular wall [2]. A systematic review and meta-analysis of observational studies found 28 eligible studies enrolling 2501 patients with left ventricular non-compaction [3]. Cardiovascular mortality was 1.92 per 100 person years at a median follow up of 2.9 years in the meta-analysis. T...
Source: Cardiophile MD - Category: Cardiology Authors: Tags: Cardiology Source Type: blogs