Abstract
The role of insulin in cardiac muscle growth has been controversial. In vitro studies have had equivocal results but insulin has been implicated as an etiologic factor in the hypertrophic cardiomyopathy seen in the infant of a diabetic mother. We have recently cared for an infant with neonatal hypoglycemia secondary to endogenous organic, hyperinsulinism (blood glucose 22mg/dl; insulin 92uU/ml) that was refractory to diazoxide, chlorothiazide and glucocorticoids. Despite a 95% pancreatectomy on day 30 which showed islet hypertrophy/hyperplasia with overall features of endocrine cell dysplasia, the hypoglycemia persisted and was marginally controlled with the same drugs. At 4 months of age, the patient presented with a severe biventricular hypertrophic cardiomyopathy and marked congestive failure (RV end diastolic pressure = 18; LV end diastolic = 23 mmHg; Septal wall thickness = 8 mm, LV free wall = 12mm) Based upon the hypothesis that hyperinsulinism might be an etiology and the refractoriness to standard anticongestive therapy, the remaining pancreas was removed. One year later there has been nearly complete resolution of the cardiomyopathy (RV end diastolic pressure = 5, LV end diastolic = 12mmHg; Septal thickness = 5mm, LV free wall = 7mm). The child is normoglycemic without medication. This patient's clinical course suggests a role for insulin in cardiac growth/hypertrophy. We recommend that infants with hyperinsulinism undergo a cardiac evaluation.
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Gutal, J., Harris, J. & Dabbs, D. HYPERINSULINISM: A REVERSIBLE CAUSE OF HYPER-TROPHIC CARDIOMYOPATHY. Pediatr Res 21 (Suppl 4), 342 (1987). https://doi.org/10.1203/00006450-198704010-01049
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DOI: https://doi.org/10.1203/00006450-198704010-01049