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A possible diagnosis of LVNC was made on the basis of echo findings.
LVNC is a rare cardiomyopathy that is characterized by the presence of a trabeculated myocardium layer, with deep inter-trabecular recesses adherent to a thin compact layer of myocardium resulting in severe left ventricular dysfunction.1The etiology of LVNC is assumed to be due to errors in embryonic development.1 First described on echocardiography in 1984 by Engberding and Bender as the persistence of isolated myocardial sinusoids.
Cardiac manifestations can include cardiomyopathies, LVNC, and arrhythmias .
It will also be interesting to conduct more research in the future to investigate mitochondriopathies to understand the link between LVNC and gastroparesis.
None of them have been consistently identified to be the single gene abnormality causing LVNC, but they are briefly included here for completeness (see the list below) .
The molecular genetics of inherited cardiomyopathies have been reviewed and the evidence to support routine genetic testing in all patients diagnosed with LVNC is not available [11,12].
The diagnosis of LVNC can be made by a transthoracic echocardiogram with contrast.
reported a case with a diagnosis of isolated non-compaction, a condition characterised by the absence of other associated cardiopathies. Although it is a well-known pathology in adults, there are only a few case reports and series in the literature regarding LVNC in children.
LVNC is an uncommon finding thought to be caused by an intrauterine arrest of normal cardiac embryogenesis (4).
We herein report the successful treatment of a 4-year-old girl who presented with incessant VF and LVNC at 5 months of age.
Cases of rare subtypes (n=15; 7 Percent), included EFE 3 (20 Percent), LVNC 3 (20 Percent), AVRC 2 (13.33 Percent), scleroderma 3 (20 Percent), SLE 1 (6.66 Percent), and muscular-
The left ventricular (LV) wall demonstrated segmental thickening with a two-layer arrangement consisting of a thin compacted epicardial layer and a thick, non-compact endocardial layer with prominent trabeculations and deep recesses comprising over 50% of the IV wall (Figure 3), consistent with LVNC. Microscopic examination of the tissue revealed focal fibrosis (Figure 4).
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