Cardiac Function in Congenital Adrenal Hyperplasia: A Pattern of Reversible Cardiomyopathy

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.