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Isolated noncompaction of ventricular myocardium—detection during fetal life

Summary Isolated noncompaction of ventricular myocardium is a rare cardiomyopathy with persistence of a myocardial architecture such as seen early in fetal development with numerous prominent trabeculations and deep intertrabecular spaces. Symptoms are related to cardiac failure, arterial embolism, and severe arrhythmias. Familial recurrence has been described. We describe a case where isolated noncompaction of ventricular myocardium could be recognized on an ultrasound study at 23 weeks gestation, thus supporting the developmental hypothesis.