ESPE Abstracts (2016) 86 P-P2-72

The ımpact of 21 Hydroxylase Deficiency on Cardiac Repolarization Changes in Children with 21-hydroxylase-deficient Congenital Adrenal Hyperplasia

Hüseyin Anıl Korkmaza, Rahmi Özdemirb, Mehmet Küçükb, Cem Karadenizb, Timur Meşeb & Behzat Özkana


aDr. Behcet Uz Children Disease and Surgery Training and Research Hospital, Division of Pediatric Endocrinology, Izmir, Turkey; bDr. Behcet Uz Children Disease and Surgery Training and Research Hospital, Division of Pediatric Cardiology, Izmir, Turkey


Background: To the best of our knowledge, no study has been conducted to assess the impact of 21 hydroxylase deficiency and hidrocortisone treatment on electrocardiographic measures in children with 21-hydroxylase-deficient congenital adrenal hyperplasia.

Objective and hypotheses: The purpose of this study is to compare the12-lead electrocardiographic measures such as PWd, QT interval, QTd, Tp-e interval, Tp-e/QT and Tp-e/QTc ratio in patients of 21-hydroxylase-deficient congenital adrenal hyperplasia with that in healthy control subjects matched for age, sex, height, weight and BMI.

Method: Twenty-five patients of 21-hydroxylase-deficient congenital adrenal hyperplasia and 25 control subjects were enrolled into this observational, cross-sectional, controlled study. The evaluation consisted of anthropometric measurements, biochemical parameters, and electrocardiographic (ECG) measures. The standard 12-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/QTc ratios were calculated.

Results: There were no significant differences in the groups for age, sex, height, weight and BMI (median age 112.8 (90.4) vs. 80.7 (109.5) months, mean weight 37.6±21.5 vs. 27.9±18.3 kg, mean height 125.4±28.9 vs. 114.7±31 cm, mean BMI 21.4±5.7 vs. 18.9±3.4 kg/m2, respectively). P dispersion and Tp-e dispersion were significantly higher in patients of 21-hydroxylase-deficient congenital adrenal hyperplasia compared to the controls (median P dispersion 50 (25) vs. 40 (40) ms, mean Tp-e dispersion 48±15.5 vs. 35.2±17.5 ms).

Conclusion: Our study revealed that P and Tp-e dispersion were increased in 21-hydroxylase deficient children. 21 hydroxylase deficiency and treatment might be risk factor for atrial and ventricular arrhythmias in children with congenital adrenal hyperplasia 21-hydroxylase-deficient.

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