Diagnosis and study of rare heart diseases: multimodality --- left ventricular non-compaction cardiomyopathy

Left ventricular non-compaction cardiomyopathy, described at the beginning of the 20th century, was considered to be of low prevalence until the 1980’s, when it was disgnosed in vivo. More recently, with the advent of new echocardiography and cardiac resonance techniques, it is diagnosed more frequently. The left ventricle is the most compromised, but the right, or both, can also be affected. The name of left ventricular non-compaction cardiomyopathy refers to myocardium has a thin layer of compacted and another layer of trabeculae with
deep intra-trabecular recesses (non-compacted myocardium), mainly at the level of the lateral wall and the apex. This is a characteristic that is the basis for determining the diagnosis both in echocardiography and cardiac resonance, with the relationship between the non-compacted myocardium and the compacted myocardium. Late gadolinium enhancement in magnetic resonance helps to identify the fibrosis, which has prognostic importance. Various phenotypes have
ben decribed that maintain the relationship with the prognosis of the disease. The arrhythmias, heart failure, sudden death, or emboli of cardiac origin are the forms that are more associated
with left ventricular non-compaction cardiomyopathy