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MÆDICA - a Journal of Clinical Medicine | Vol. 9, nr. 3, 2014

ISSN 1841-9038  |  e-ISSN 2069-6116
ISSN-L 1841-9038
CNCSIS - CMR - B+

Left Ventricular Non-Compaction – Challenges and Controversies

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ABSTRACT

Cardiomyopathies classification is based on morphological and functional phenotypes and subcategories of familial/genetic and non-familial/non-genetic disease. The non-compaction cardiomyopathy is a rare disorder which is considered to be an unclassified cardiomyopathy according to the ESC Working Group on Myocardial and Pericardial Diseases and the World Health Organization or a primary genetically-determined cardiomyopathy according to the American Heart Association. The diagnosis of non-compaction is challenging and its nosology is debated since this morphological trait can be shared by different cardiomyopathies and non-cardiomyopathy conditions. Myocardial structure has a spectrum from normal variants to the pathological phenotype of non-compaction cardiomyopathy, which reflects the embryonic structure of the human heart due to an arrest in the compaction process during the first trimester. However, when a definite diagnosis of non-compaction is made, the diagnostic process should orient towards a genetic disease with a relatively high probability of sarcomere mutations. Non-compaction cardiomyopathy is a diagnostically challenging entity. Nowadays there are some controversies associated with this cardiomyopathy, that it worth to be discussed.

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