LEFT VENTRICULAR NONCOMPACTION

The left non compacted ventricle is a form of nonclassifiable myocardiopathy. It occurs in the fetal stage, caused by a detention of the normal process of compactation of the ventricular wall, presenting a large trabecular layer, with multiple recesses on the endocavity side. It is possible to identify isolated and familiar forms (20%-50% of the cases) due to genetic heterogeneity with documented proof of diverse genetic mutations. Clinically, it appears through cardiac insufficiency, arrhytmias and systemic thromboembolism. There is no clinical case of severe cardiac insufficiency. The diagnosis by echocardiography reveals a relationship between the thick, non compacted leyer and the compacted myocardium > 2, being possible to visualize the blood flow by color Doppler, in the prominent intertrabecular separations. The cardiac magnetic resonance diagnosis, through the sequence called "steady state free precession". Nowadays, there is no specific treatment. The cardiac insufficiency and the complications are theated; in the future, the genetic therapy will be promissory though.