title: Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors: Division 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.
authors: TingTing
authors: T
authors: Huang
authors: Andrea
authors: A
authors: Kelly
authors: Susan A
authors: SA
authors: Becker
authors: Meryl S
authors: MS
authors: Cohen
authors: Charles A
authors: CA
authors: Stanley
mesh_terms: D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000378:metabolism; D015331:Cohort Studies; D044903:Congenital Hyperinsulinism / Q000150:complications* / Q000378:metabolism; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D007328:Insulin / Q000378:metabolism; D054086:KATP Channels / Q000378:metabolism; D015143:Philadelphia; D012189:Retrospective Studies; D012307:Risk Factors
chemical_list: D007328:Insulin; D054086:KATP Channels
keywords: Cardiology; Endocrinology
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