Left Ventricular Noncompaction Cardiomyopathy (LVNC) Panel

Left ventricular noncompaction (LVNC) or 'spongy myocardium' is a rare congenital cardiomyopathy that can be diagnosed at any age. It is defined by three echocardiographic markers: prominent left ventricular (LV) trabeculae, deep intertrabecular recesses, and the thin compacted layer. Based on echocardiographic studies, reported prevalence is between 0.014% and 1.3% in the general population

It has been classified as a primary cardiomyopathy of genetic origin that usually affects the left ventricle; however, right ventricular and biventricular non-compaction have also been described. More than half of the genetic defects are found in genes responsible for sarcomere function (e.g: MYBPC3 or MYH7), which are crucial for heart muscle contraction. Sarcomeres are the basic contractile unit of muscle fiber, and they are composed of two main protein filaments (thin actin and thick myosin filaments). LVNC is often inherited in an autosomal dominant manner, but autosomal recessive and X-linked inheritance patterns also occur.