Cardiac function in congenital adrenal hyperplasia

A pattern of reversible cardiomyopathy

Mary Minette, Andrew W. Hoyer, Phat P. Pham, Mark D. Deboer, Mark Reller, Bruce Boston

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
JournalJournal of Pediatrics
Volume162
Issue number6
DOIs
StatePublished - Jun 2013

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Congenital Adrenal Hyperplasia
Cardiomyopathies
Adrenal Cortex Hormones
Blood Pressure
Control Groups
Therapeutics
Newborn Infant
Echocardiography
Case-Control Studies

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Cardiac function in congenital adrenal hyperplasia : A pattern of reversible cardiomyopathy. / Minette, Mary; Hoyer, Andrew W.; Pham, Phat P.; Deboer, Mark D.; Reller, Mark; Boston, Bruce.

In: Journal of Pediatrics, Vol. 162, No. 6, 06.2013.

Research output: Contribution to journalArticle

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abstract = "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.",
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N2 - 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.

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