Left ventricular non compaction: epidemiology, clinical findings, diagnosis and therapy

Left ventricular non compaction (LVNC) is a cardiomyopathy due to an arrest of the normal development of myocardium which determines the persistence of fetal myocardium in postnatal life in at least 2/3 of the wall (criterion known as non compacted/ compacted ratio greater than 2). Although in absence of a confirmed prevalence of LVNC, reviewing literature shows an increasing number of reports over the years, though diagnosed cases represent just the tip of a realistically far wider phenomenon. Clinical manifestations are variable, ranging from the absence of any symptom to congestive heart failure, arrhythmias and systemic thromboembolism. Echocardiography is the gold standard for the diagnosis. Tissue Doppler and three-dimensional echocardiography may give further information in the evaluation of patients affected by LVNC. Magnetic resonance could refine diagnosis particularly in those patients with not conclusive echocardiogram: it may help in differential diagnosis and give prognostic information. There is no specific therapy for patients with LVNC but the treatment is aimed at treating heart failure, or other complications such as arrhythmias and thromboembolic events.