title: Cardiac function in congenital adrenal hyperplasia: a pattern of reversible cardiomyopathy.
abstract: OBJECTIVE
  To evaluate cardiac function in infants with congenital adrenal hyperplasia (CAH) before and after corticosteroid replacement therapy.
  
  
  METHODS
  This prospective, case-control study included 9 infants with CAH. Cardiac function was assessed by echocardiography at presentation and after corticosteroid replacement therapy. Six term infants underwent 2 echocardiograms each and served as the control group. Data on fractional shortening (FS), rate-corrected velocity of circumferential fiber shortening (Vcf), wall stress, tissue Doppler indices, myocardial performance index, left ventricular mass, and Vcf/wall stress were obtained.
  
  
  RESULTS
  The infants with CAH exhibited myocardial dysfunction at baseline and lower systolic blood pressure (SBP) compared with the control group. FS, a measure of systolic contractility, differed significantly from before to after corticosteroid treatment (mean, 32.3%±4.7% pretreatment, 39.9%±5.0% posttreatment). Vcf, a preload-independent measure of cardiac contractility, also differed significantly before and after treatment (mean, 1.23±0.16 circumferences/second pretreatment, 1.45±0.22 circumferences/second posttreatment). SBP was also lower (mean, 84±9.3 mmHg) and improved with treatment (mean, 95±4.8 mmHg). The control group demonstrated no statistically significant changes in FS, Vcf, or SBP. There was a change in left ventricular mass in the control group between the 2 studies.
  
  
  CONCLUSIONS
  Newborns with CAH have evidence for cardiac dysfunction at baseline that reverses with corticosteroid replacement therapy. These data suggest that corticosteroids play a direct role in modulating cardiac function in the newborn.
authors: Division of Pediatric Cardiology, Department of Pediatrics, Oregon Health & Science University, Portland, OR 97239, USA. minettem@ohsu.edu
authors: Mary S
authors: MS
authors: Minette
authors: Andrew W
authors: AW
authors: Hoyer
authors: Phat P
authors: PP
authors: Pham
authors: Mark D
authors: MD
authors: DeBoer
authors: Mark D
authors: MD
authors: Reller
authors: Bruce A
authors: BA
authors: Boston
mesh_terms: D000305:Adrenal Cortex Hormones / Q000494:pharmacology* / Q000627:therapeutic use; D000312:Adrenal Hyperplasia, Congenital / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001794:Blood Pressure; D009202:Cardiomyopathies / Q000000981:diagnostic imaging / Q000503:physiopathology*; D016022:Case-Control Studies; D004452:Echocardiography; D005260:Female; D020249:Hormone Replacement Therapy / Q000379:methods*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009200:Myocardial Contraction; D011446:Prospective Studies; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000503:physiopathology*
chemical_list: D000305:Adrenal Cortex Hormones