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This 49-day-old term male baby was born to a healthy mother by normal spontaneous delivery weighing 3260 g. Tachypnoea with subcostal retraction, gasping, general malaise, and poor appetite were noted from the age of 14 days. Echocardiography revealed hypertrophic cardiomyopathy with systolic dysfunction. The left ventricular ejection fraction was 35%. He had no familial history of cardiovascular disease. Contrast enhanced cardiac computed tomography (LightSpeed VCT, General Electric Medical Systems, Milwaukee, Wisconsin, USA) revealed severe thickening of the myocardium with prominent trabeculations and deep intertrabecular recesses throughout the left ventricular walls (panel A). The thickness of the compacted layer was 22% of the left ventricular lateral wall. Virtual cardioscopy (Advantage Windows 4.3; General Electric Medical Systems, Paris, France) along the axis from the mitral inlet through to the LV apex well demonstrated the interlaced myocardial bundles in the septal, apical and lateral walls of the left ventricle (panel B; movies 1 and 2).
This article has been adapted from Chen S-J, Lee W-J, Lin M-T. Left ventricular non-compaction in an infant Heart 2008;94:295
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