Tuhan Hale, Demircan Tülay, Altıncık Ayca, Çatlı Gönül, Kızılca Özgür, Egeli Tuğba, Kır Mustafa, Can Şule, Dündar Bumin, Böber Ece, Abacı Ayhan
1Department of Pediatric Endocrinology,Dokuz Eylül University,Faculty of Medicine,Izmir,Turkey.
2Department of Pediatric Cardiology,Dokuz Eylül University,Faculty of Medicine,Izmir,Turkey.
Cardiol Young. 2019 Mar;29(3):319-324. doi: 10.1017/S1047951118002330. Epub 2019 Jan 24.
AimThe present study aimed to evaluate systolic and diastolic myocardial function in children and adolescents with congenital adrenal hyperplasia.
The study included 44 children with the diagnosis of classic congenital adrenal hyperplasia and 39 healthy children whose age, pubertal status, and gender were similar to those of the patient group. Anthropometric parameters and 17-hydroxyprogesterone levels were measured, and bone age was calculated. The average daily hydrocortisone dose was calculated over the last 1-year file records. Hyperandrogenic state was defined according to bone age SD score (⩾2) and 17-hydroxyprogesterone levels (>10 ng/ml). Echocardiographic examinations were assessed by conventional two-dimensional Doppler echocardiography and tissue Doppler imaging.
Patients had higher morphological parameters, such as left ventricular end-systolic diameter, interventricular septal thickness at end diastole, left ventricular posterior wall thickness at end diastole, left ventricular mass and index, than the control group (p<0.05). On pulsed-wave and tissue Doppler echocardiography, significant subclinical alterations were observed in systolic (isovolumic contraction time), diastolic (isovolumic relaxation time), and global left ventricular functional (myocardial performance index) parameters in the congenital adrenal hyperplasia group compared to the control group (p<0.05). In partial correlation analyses, after controlling the effect of hyperandrogenism, the mean hydrocortisone dosage was positively correlated with isovolumic relaxation time in congenital adrenal hyperplasia group (p<0.05).
This study demonstrated that the patients with congenital adrenal hyperplasia are at risk for left ventricular hypertrophy, systolic and diastolic myocardial subclinical alterations. Overtreatment may be responsible for the increased risk of myocardial dysfunction in patients with congenital adrenal hyperplasia.
目的
本研究旨在评估先天性肾上腺皮质增生症患儿及青少年的收缩期和舒张期心肌功能。
本研究纳入了44例诊断为经典型先天性肾上腺皮质增生症的患儿以及39例年龄、青春期状态和性别与患者组相似的健康儿童。测量人体测量学参数和17-羟孕酮水平，并计算骨龄。根据过去1年的病历记录计算氢化可的松的平均每日剂量。根据骨龄标准差评分（⩾2）和17-羟孕酮水平（>10 ng/ml）定义高雄激素状态。通过传统二维多普勒超声心动图和组织多普勒成像评估超声心动图检查结果。
与对照组相比，患者的形态学参数更高，如左心室收缩末期内径、舒张末期室间隔厚度、舒张末期左心室后壁厚度、左心室质量和指数（p<0.05）。在脉冲波和组织多普勒超声心动图检查中，与对照组相比，先天性肾上腺皮质增生症组在收缩期（等容收缩时间）、舒张期（等容舒张时间）和左心室整体功能（心肌性能指数）参数方面观察到明显的亚临床改变（p<0.05）。在偏相关分析中，在控制高雄激素血症的影响后，先天性肾上腺皮质增生症组的氢化可的松平均剂量与等容舒张时间呈正相关（p<0.05）。
本研究表明，先天性肾上腺皮质增生症患者存在左心室肥厚、收缩期和舒张期心肌亚临床改变的风险。过度治疗可能是先天性肾上腺皮质增生症患者心肌功能障碍风险增加的原因。
