Left Ventricular Noncompaction is a genetic cardiac disease requiring echocardiographic diagnosis and screening of first-degree relatives due to its familial association.
Supports relative screening in LVNC; leaves open optimal protocols and outcome impact pending prospective data.
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.
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Malla et al. (2009) studied this question.
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