engleski

Isolated left ventricular noncompaction

Isolated left ventricular noncompaction (LVNC) is a relatively new entity that is still very often not recognised. In most cases it is caused by developmental disturbance in endomyocardial morphogenesis, and in rare cases probably by regression of adult into embryonic structure. Clinical presentation includes systolic dysfunction of the left ventricle in more than half of the cases, ventricular arrhythmia and embolism. It is often accompanied by neuromuscular disorders. The paper describes three pts (two female and one male patient ; ages 49, 60, and 49) with the isolated LVNC. In all the pts the illness presented itself in limitations regarding physical activity. The pts presented the typical cases of isolated LVNC that met all 3 diagnostic criteria (typical location, noncompacted to compacted layer ratio >2, colour Doppler imaging demonstrating intertrabecular recesses communicating with the LV cavity). All pts presented with the symptoms of dilated cardiomyopathy. None of the pts had the associated neuromuscular disorder. Since there were no other family members with similar disorders, these were probably the first mutants.

heart; left ventricle; development; noncompaction

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