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Left ventricular non-compaction (LVNC) is a genetic cardiomyopathy often familial and autosomal dominant. It is characterized by morphological abnormalities affecting the left ventricular myocardium with prominent trabeculations of the inner surface of the ventricle, often extending deep into the ventricular wall. There are no pathognomonic histological findings with normal myocytes being interspersed with areas of fibrosis. Both familial and sporadic forms of non-compaction have been described, the prevalence being estimated to be between 0.01% and 0.27%.1 The diagnosis of LVNC calls for multimodal imaging. Echocardiography, being the most widely available cardiac imaging modality, may raise the initial suspicion of LVNC and can also provide physiological data. See Table 1. Cardiac Magnetic Resonance Imaging (CMR) offers detailed visualization of the extent of non-compaction and supplemental morphological information.4 It should be noted that the current criteria for this condition may result in over diagnosis. View this table:Table 1 Criteria for the diagnosis of isolated left ventricular non-compactionA correct diagnosis is important both for subsequent treatment and also to enable appropriate genetic counselling and familial screening. It is suggested that first degree relatives should be screened. CMR is the best imaging modality currently, though other techniques in the future may be able to improve on diagnostic accuracy. LVNC is associated with LV systolic dysfunction, due to subendocardial hypoperfusion and microcirculation dysfunction, and at a lesser extent to diastolic dysfunction, ventricular arrhythmias, sudden cardiac death and systemic embolism.There is no specific treatment for LVNC at present, with the …

Treatment of left ventricular non-compaction with cardiac resynchronization therapy