Ventricular muscle abnormalities are not infrequent. They present in the setting of congenital heart disease or with a structurally normal heart. The diagnosis of left ventricular non-compaction (LVNC) can be made by echocardiography, CT, MRI or during angiography at the time of cardiac catheterization. The widely accepted diagnostic criteria are a 2-to-1 ratio of non-compacted myocardium to compacted ventricular muscle. There may be a genetic basis for this disorder as a few gene abnormalities have been identified. The purpose of this study is to detail the various clinical presentations of this condition. We present 7 patients with left ventricular non-compaction cardiomyopatlry, all of whom had different clinical presentations. The outcomes varied widely ranging from asymptomatic to awaiting heart transplant to death.Conclusion: LVNC is a serious abnormality of the myocardium and has a diverse clinical manifestation. The pediatric community needs to be aware of this disorder and be ready to appropriately manage these patients.
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