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Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism

TingTing Huang1, Andrea Kelly, Susan A Becker

  • 1Division of Endocrinology & Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, 34th and Civic Center Boulevard, Philadelphia, PA 19104, USA.

Archives of Disease in Childhood. Fetal and Neonatal Edition
|February 5, 2013
PubMed

Insights

Hypertrophic cardiomyopathy (HCM) is common in infants with severe congenital hyperinsulinism. Early echocardiograms are recommended for at-risk newborns to identify this complication.

Area of Science:

  • Pediatrics
  • Cardiology
  • Endocrinology

Background:

  • Hypertrophic cardiomyopathy (HCM) is a known complication in infants of diabetic mothers, linked to increased fetal insulin. Congenital hyperinsulinism (HI) involves excessive insulin secretion due to genetic defects, such as KATP channel mutations.
  • While HCM has been observed in a few neonates with HI, its prevalence and risk factors remain unevaluated.

Purpose of the Study:

  • To evaluate the extent and risk factors for hypertrophic cardiomyopathy (HCM) in infants with congenital hyperinsulinism (HI).

Main Methods:

  • A retrospective chart review was conducted on infants under 3 months old with congenital hyperinsulinism treated at Children's Hospital of Philadelphia over 3.5 years.
  • Data collected included gestational age, birth weight, HI type and treatment, and cardiac and respiratory outcomes, with a focus on echocardiogram results.

Main Results:

  • Of 68 infants with HI, 25 underwent echocardiography, revealing 10 cases of HCM. All 10 infants with HCM required pancreatectomy, and 8 had confirmed ATP-sensitive potassium-hyperinsulinism.
  • Infants with HCM were born at an earlier gestational age (36 weeks) compared to those without HCM (38 weeks) (p=0.02).

Conclusions:

  • Hypertrophic cardiomyopathy (HCM) appears to be a common complication in infants with severe congenital hyperinsulinism (HI).
  • Routine echocardiograms and EKGs are recommended for newborns at risk of HI.
  • Excessive fetal insulin secretion is the likely cause of HCM in infants with hyperinsulinism.
Abstract

Keywords:
CardiologyEndocrinology

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