Cardiac function in children with congenital adrenal hyperplasia

Glucocorticoid excess or deficiency with hyperandrogenism may cause cardiovascular morbidity in patients with congenital adrenal hyperplasia (CAH) due to failing to mimic physiological circadian rhythm. This study aimed to evaluate the cardiac function in pediatric patients with CAH treated with conventional hydrocortisone treatment and its relation to hydrocortisone dose and therapy duration.

Twenty-three pediatric patients with CAH, aged 3-16 years, and 21 age- and gender-matched healthy controls were enrolled to the study. All the patients were evaluated by physical examination, electrocardiogram (ECG), conventional echocardiography, and tissue Doppler imaging (TDI).

The mean Sm (systolic myocardial velocity) in the CAH group was 7.96 ± 1.22 cm/s, which was significantly lower than that in the control group (p=0.034). The mean systolic excursion in the CAH group was 14.17 ± 1.92 mm, which was significantly lower than that in the control group (p=0.046). The tricuspid-derived early diastolic myocardial velocity (Em) and the tricuspid-derived E/Em ratio were significantly higher in the CAH group than in the control group (p=0.003 and 0.008, respectively). No signs of left ventricular hypertrophy or dilatation were detected on ECG and echocardiography.

Long-term conventional hydrocortisone therapy even within the recommended therapeutic range may adversely affect cardiac functions in children with 21-hydroxylase deficiency.