Isolated non-compaction cardiomyopathy is a rare disease that is likely to 
develop in the embryonic period. It is caused by the intrauterine arrest of the 
myocardial compaction process in the beginning of the fetal development. It is 
characterized by prominent myocardial trabeculations and deep intertrabecular 
recesses, as well as the thickening of the myocardium into two distinct layers 
(compacted and not compacted). Even though this disease is said to be prevalent 
in the pediatric population or together with congenital heart disease, one can 
understand that this disease occurs in isolation, because the diagnosis is 
becoming more common in adult patients that have no other heart disease. The 
clinical manifestations vary greatly, because they range from absence of 
symptoms to congestive heart failure, arrhythmias and systemic thromboembolism. 
Echocardiography is the most widely used diagnostic procedure, but the little 
knowledge about this disease, its similarity to other myocardial diseases and 
the limitation of the echocardiographic technique used delay the diagnosis. The 
purpose of this review is to show that that other imaging techniques, such as 
MRI, CT and left ventriculography have emerged as diagnostic alternatives.