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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., (Copyright © 2013 Mosby, Inc. All rights reserved.)

An 11 day-old female infant underwent resection of a mass in the subaortic region secondary to critical aortic stenosis. At 3 months of age, recurrent severe left-ventricular outflow obstruction (LVOT) in the setting of heart failure prompted redo surgery, and the resected mass revealed an intracardiac foregut cyst, which is a rare finding. To our knowledge, this is the first case describing obstruction of the LVOT.

Background: Invasive monitors and noninvasive 2-dimensional echocardiography are the standard clinical methods for stroke volume (SV) and cardiac output computation. We studied the use of real-time color Doppler 3-dimensional (3D) echocardiography (3DE) for the assessment of SV in human beings., Methods: In all, 55 pediatric and adult patients with good transthoracic windows and a normal aortic valve were studied. Real-time 3DE color Doppler volumes incorporating the left ventricular outflow tract and aortic valve were taken. SV was calculated from the color Doppler data in the 3DE DICOM dataset. This was compared with 2-dimensional echocardiography SV calculation from the pulsed wave velocity through the aortic valve along with the left ventricular outflow tract diameter., Results: Five patients were excluded because of mismatching of the 3D color Doppler segments in the 3D volume. The 3D Doppler volumes from the remaining 50 patients were analyzed. There was good correlation between the patients' averaged 3DE SV calculations and the 2-dimensional echocardiography pulsed wave SV estimation (y = 0.84x + 7.8, r2 = 0.90)., Conclusion: Real-time 3D Doppler echocardiography can be used to accurately calculate SV and cardiac output, compared with conventional pulsed Doppler measurement, in pediatric and adult patients from transthoracic imaging.