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

• Published in: The Journal of pediatrics Vol. 162; no. 6; pp. 1193 - 1198.e1
• Main Authors: Minette, Mary S., MD, Hoyer, Andrew W., MD, Pham, Phat P., MD, DeBoer, Mark D., MD, Reller, Mark D., MD, Boston, Bruce A., MD
• Format: Journal Article
• Language: English
• Published: United States Mosby, Inc 01.06.2013
• Subjects: adrenal cortex hormones; Adrenal Cortex Hormones - pharmacology; Adrenal Cortex Hormones - therapeutic use; Adrenal Hyperplasia, Congenital - diagnostic imaging; Adrenal Hyperplasia, Congenital - physiopathology; Blood Pressure; cardiac output; Cardiomyopathies - diagnostic imaging; Cardiomyopathies - physiopathology; cardiomyopathy; Case-Control Studies; Echocardiography; Female; Hormone Replacement Therapy - methods; Humans; hyperplasia; Infant; Infant, Newborn; Male; Myocardial Contraction; neonates; Pediatrics; Prospective Studies; systolic blood pressure; therapeutics; Ventricular Dysfunction, Left - diagnostic imaging; Ventricular Dysfunction, Left - physiopathology; Fractional shortening; SBP; Systolic blood pressure; MPI; V cf; CAH; Congenital adrenal hyperplasia; Myocardial performance index; FS; Rate-corrected velocity of circumferential fiber shortening; Vcf
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2012.11.086

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 (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. 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.