Abstract
Left Ventricular Noncompaction (LVNC) is a genetic cardiac disease of emerging importance with a distinct clinical and pathophysiological presentation. The most common clinical manifestations include heart failure, arrhythmias and embolic events, and in children it may be associated with facial dysmorphisms and Wolff-Parkinson-White syndrome. The diagnosis of LVNC, however, is often missed, most often as a consequence of ignorance of the condition. Echocardiography is considered the reference standard for the diagnosis of LVNC. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. Because of the familial association of LVNC, first-degree relatives should be screened by Echocardiography.
Highlights
The persistence of prominent myocardial trabeculation, seen in the normal right ventricle, does not usually occur after birth in the left ventricle.[1]
During the early embryonic phase, the left ventricular myocardium consists of a loose network of fibres separated by deep recesses that link the myocardium with the left ventricular cavity
Malla et al Left Ventricular Noncompaction died of acute left heart failure, an autopsy proved for the first time the diagnosis of persistent sinusoids, today called LVNC.[8,9]
Summary
The persistence of prominent myocardial trabeculation, seen in the normal right ventricle, does not usually occur after birth in the left ventricle.[1] During the early embryonic phase, the left ventricular myocardium consists of a loose network of fibres separated by deep recesses that link the myocardium with the left ventricular cavity. Gradual compaction of these fibres and intertrabecular recesses usually occurs within 8 weeks of life, progressing from the epicardium to endocardium and from the base of the heart to the apex.[1]. Malla et al Left Ventricular Noncompaction died of acute left heart failure, an autopsy proved for the first time the diagnosis of persistent sinusoids, today called LVNC.[8,9]
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