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Cardiac function in congenital adrenal hyperplasia: a pattern of reversible cardiomyopathy

Mary S Minette1, Andrew W Hoyer, Phat P Pham

  • 1Division of Pediatric Cardiology, Department of Pediatrics, Oregon Health & Science University, Portland, OR 97239, USA. minettem@ohsu.edu

The Journal of Pediatrics
|January 23, 2013
PubMed

Insights

Newborns with congenital adrenal hyperplasia (CAH) show cardiac dysfunction that improves with corticosteroid therapy. This suggests corticosteroids directly impact newborn heart function.

Area of Science:

  • Pediatric Endocrinology
  • Neonatal Cardiology
  • Pediatric Cardiology

Background:

  • Congenital adrenal hyperplasia (CAH) can affect multiple organ systems.
  • Cardiac function in infants with CAH requires further investigation.
  • The impact of corticosteroid replacement on neonatal cardiac health is not fully understood.

Purpose of the Study:

  • To assess cardiac function in infants diagnosed with congenital adrenal hyperplasia (CAH).
  • To evaluate the effects of corticosteroid replacement therapy on cardiac parameters in infants with CAH.
  • To compare cardiac function in infants with CAH to a healthy control group.

Main Methods:

  • Prospective case-control study involving 9 infants with CAH and 6 healthy term infants.
  • Echocardiography was performed at presentation and after corticosteroid replacement therapy in infants with CAH.
  • Cardiac function was assessed using fractional shortening (FS), velocity of circumferential fiber shortening (Vcf), wall stress, and other echocardiographic indices.

Main Results:

  • Infants with CAH presented with myocardial dysfunction and lower systolic blood pressure (SBP) compared to controls.
  • Fractional shortening (FS) and Vcf significantly improved after corticosteroid treatment in infants with CAH.
  • Systolic blood pressure (SBP) also increased significantly following corticosteroid therapy in the CAH group.

Conclusions:

  • Newborns with CAH exhibit cardiac dysfunction at baseline.
  • Corticosteroid replacement therapy effectively reverses cardiac dysfunction in infants with CAH.
  • These findings indicate a direct role for corticosteroids in modulating cardiac function in newborns.
Abstract

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