Hypertrophic cardiomyopathy (HCM) associated with diabetes during pregnancy has been relatively recently described. The echocardiographic findings are similar to those found in the classic sarcomeric familial HCM. In addition, the hemodynamics and clinical presentation may be significant and can include impaired cardiac output and pulmonary hypertension as a result of diastolic dysfunction, left ventricular outflow tract obstruction, and systolic dysfunction. Unlike sarcomeric HCM, neonatal HCM associated with diabetes during pregnancy is self-limited and in fact tends to resolve in weeks to months. On the other hand, in the impaired neonate, a rational management plan needs to be instituted. This article describes the history, potential causes, and pathophysiology of HCM and details a rational management plan for the treatment of these patients.

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