Cardiac function in congenital adrenal hyperplasia: a pattern of reversible cardiomyopathy.
The Journal of Pediatrics(2013)
摘要
Objective To evaluate cardiac function in infants with congenital adrenal hyperplasia (CAH) before and after corticosteroid replacement therapy. Study design 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 (V-cf), wall stress, tissue Doppler indices, myocardial performance index, left ventricular mass, and V-cf/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). V-cf, 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, V-cf, or SBP. There was a change in left ventricular mass in the control group between the 2 studies. Conclusion 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.
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关键词
CAH,FS,MPI,SBP,Vcf
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