Left Ventricular Non-compaction

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  • Left ventricular non-compaction (LVNC) is a recently recognised, rare form of cardiomyopathy. It is characterised by abnormal trabeculation, most commonly at the apex, and is often associated with ventricular hypertrophy, dilation or impairment of function. LVNC is associated with several mutations and may have a genetic overlap with other cardiomyopathic phenotypes including hypertrophic cardiomyopathy.

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    Left ventricular non-compaction (LVNC) is a recently recognised, rare form of cardiomyopathy. It is characterised by abnormal trabeculation, most commonly at the apex, and is often associated with ventricular hypertrophy, dilation or impairment of function. LVNC is associated with several mutations and may have a genetic overlap with other cardiomyopathic phenotypes including hypertrophic cardiomyopathy.

    The molecular mechanisms of LVNC are not yet fully understood but a genetic link is reported in 30–50 % of patients. On cardiac MRI, a non-compacted to compacted myocardium ratio of >2.3 has been proposed as a diagnostic criterion for LVNC.

    Doppler echocardiogram is the diagnostic procedure of choice and treatment is symptomatic management of the symptoms and complications. Early diagnosis and treatment is required as these patients can have fatal outcomes from the arrhythmias, thromboembolic phenomena and heart failure. Aggressive management is required for high-risk patients that include symptomatic treatment as well as ICD placement and possible transplant, if feasible.

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