The impact of 21-hydroxylase deficiency on cardiac repolarization changes in children with 21-hydroxylasedeficient congenital adrenal hyperplasia

摘要

21-hydroxylase-deficient congenital adrenal hyperplasia (CAH) is associatedwith cardiovascular risk factors such as, hypertension, obesity, dyslipidemia,and insulin resistance. It is not known whether 21-hydroxylase-deficient CAHis risk factor for atrial and ventricular arrhythmias. The purpose of this studywas to compare the 12-lead electrocardiographic measures in patients of21-hydroxylase-deficient congenital adrenal hyperplasia with those in healthycontrol subjects matched for age, sex, height, weight and body mass index(BMI). Twenty-five patients with 21-hydroxylase-deficient CAH and twenty-fiveheathy control subjects were enrolled into this observational, cross-sectional,controlled study. The evaluation consisted of anthropometric measurements,biochemical parameters, and electrocardiographic (ECG) measures. The standard12-lead electrocardiography was performed in all patients and P-wave dispersion(PWd), QT interval, QTd, QTcd, Tp-e dispersion, Tp-e/QT and Tp-e/QTcratios were calculated. There were no significant differences in the groups forage, sex, height, weight and BMI (median age 9.4 (1.5–16.75) years, meanweight 37.6±21.5 vs. 27.9±18.3 kg, mean height 125.4±28.9 vs. 114.7±31cm, mean BMI 21.4±5.7 vs. 18.9±3.4 kg/m2, respectively). P dispersion andTp-e dispersion were significantly higher in patients of 21-hydroxylase-deficientCAH compared to the healthy subjects (median P dispersion 50 (25) vs.40 (40) ms, mean Tp-e dispersion 48±15.5 vs. 35.2±17.5 ms). Our studyrevealed that 21-hydroxylase deficient CAH is associated with high risk ofatrial and ventricular arrhythmias in children.