Journal
CURRENT OPINION IN CARDIOLOGY
Volume 24, Issue 3, Pages 209-213Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/HCO.0b013e32832a11e7
Keywords
cardiomyopathies; left ventricular noncompaction; sarcomeric proteins
Categories
Ask authors/readers for more resources
Purpose of review Isolated left ventricular noncompaction (LVNC) is a myocardial disorder characterized by excessive and prominent trabeculations of the left ventricle, associated with progressive systolic failure, stroke and arrhythmia. Until quite recently, LVNC was thought to be extremely rare, but, with greater awareness of the disease and improvements in echocardiographic technology, there has been a dramatic increase in the frequency of diagnosis. Recent studies suggest that the frequency of LVNC is determined in part by the diagnostic criteria used. Recent findings Up to 50% of adult patients with LVNC have mutations in genes encoding proteins of the cardiac sarcomere, suggesting that LVNC might represent a new disease paradigm in which mutations that more typically cause dilated and hypertrophic cardiomyopathies result in abnormal ventricular morphogenesis. Summary In this review, we briefly summarize current clinical literature on LVNC, with a particular focus on the limitations of current diagnostic criteria and emerging data on the genetics of the disorder.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available