cardiomyopathies/physiology/etiology/genetics; arrhythmia; pulmonary edema/etiology; echocardiography; spongiform cardiomyopathy; LVNC; inborn genetic diseases


Cardiology | Cardiovascular Diseases | Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Internal Medicine


Left ventricular non-compaction cardiomyopathy is an uncommon type of cardiomyopathy caused by malformation of the myocardium during embryogenesis. This results in trabeculations within the ventricular wall that can affect the left and, less commonly, right ventricles. Presentation ranges from clinically asymptomatic to life-threatening arrhythmias. It is a rare and relatively unknown form of cardiomyopathy, though thought to be underdiagnosed. Prevalence is increasing due to improvements in imaging and awareness. Management is similar to that of other cardiomyopathies including angiotensin-converting-enzyme inhibitors or angiotensin receptor blockers, beta-blockers, diuretics, automatic implantable cardioverter defibrillator placement and cardiac transplantation. We present a case of a 38-year-old, otherwise healthy, Indian male who presented with flash pulmonary edema and was found to have left ventricular non-compaction cardiomyopathy. This report includes a review of left ventricular non-compaction cardiomyopathy.

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Figure 1. Parasternal short axis view

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Figure 2. Apical long axis view

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Figure 3. Apical 2-chamber view

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Figure 4. Apical 4-chamber view

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Figure 5. Apical 4-chamber view