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Arch Dis Child Fetal Neonatal Ed doi:10.1136/archdischild-2012-302546
  • Original article

Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism

  1. Charles A Stanley2
  1. 1Division of Endocrinology & Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
  2. 2Division of Endocrinology & Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
  3. 3Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Andrea Kelly, Division of Endocrinology & Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, 11NW Main building, 34th and Civic Center Boulevard, Philadelphia, PA 19104, USA; kellya{at}email.chop.edu
  • Received 22 June 2012
  • Revised 18 November 2013
  • Accepted 4 January 2013
  • Published Online First 1 February 2013

Abstract

Introduction Hypertrophic cardiomyopathy (HCM) is a well-recognised complication in infants of diabetic mothers and is attributed to a compensatory increase in fetal insulin secretion. Infants with congenital hyperinsulinism have excessive prenatal and postnatal insulin secretion due to defects in pathways of insulin secretion (most commonly the KATP channel). HCM has been reported in a few neonates with hyperinsulinism, but its extent and risk factors for its development have not been evaluated.

Methods Retrospective chart review of infants, age <3 months, with congenital hyperinsulinism managed by Children's Hospital of Philadelphia over a 3.5-year period.

Data Gestational age, birth weight, hyperinsulinism form and treatments, echocardiogram results, cardiac/respiratory complications.

Results 68 infants were included, 58 requiring pancreatectomy for diffuse (n=28) or focal (n=30) disease, 10 were diazoxide-sensitive. Twenty-five had echocardiograms performed. Ten had HCM, all of whom required pancreatectomy and eight of whom had confirmed ATP-sensitive potassium-hyperinsulinism. Subjects with HCM had younger gestational age 36(32, 38) than their surgical counterparts without HCM 38 (31.6, 43), p=0.02.

Discussion HCM appears common in infants with severe hyperinsulinism. Routine echocardiogram and EKG of at-risk newborns should be considered. Fetal hyperinsulinism is the likely mediating factor for HCM in HI infants.

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