Department of Pediatrics, Sungae Hospital, Seoul, Korea.
J Korean Med Sci. 2013 Aug;28(8):1169-73. doi: 10.3346/jkms.2013.28.8.1169. Epub 2013 Jul 31.
Turner syndrome is well known to be associated with significant cardiovascular abnormalities. This paper studied the incidence of cardiovascular abnormalities in asymptomatic adolescent patients with Turner syndrome using multidetector computed tomography (MDCT) instead of echocardiography. Twenty subjects diagnosed with Turner syndrome who had no cardiac symptoms were included. Blood pressure and electrocardiography (ECG) was checked. Cardiovascular abnormalities were checked by MDCT. According to the ECG results, 11 had a prolonged QTc interval, 5 had a posterior fascicular block, 3 had a ventricular conduction disorder. MDCT revealed vascular abnormalities in 13 patients (65%). Three patients had an aberrant right subclavian artery, 2 had dilatation of left subclavian artery, and others had an aortic root dilatation, aortic diverticulum, and abnormal left vertebral artery. As for venous abnormalities, 3 patients had partial anomalous pulmonary venous return and 2 had a persistent left superior vena cava. This study found cardiovascular abnormalities in 65% of asymptomatic Turner syndrome patients using MDCT. Even though, there are no cardiac symptoms in Turner syndrome patients, a complete evaluation of the heart with echocardiography or MDCT at transition period to adults must be performed.
特纳综合征众所周知与显著的心血管异常相关。本文使用多排螺旋 CT（MDCT）而非超声心动图研究了无症状青少年特纳综合征患者的心血管异常发生率。纳入了 20 名无心脏症状的特纳综合征患者。检查血压和心电图（ECG）。使用 MDCT 检查心血管异常。根据 ECG 结果，11 人 QTc 间期延长，5 人出现后束支阻滞，3 人出现心室传导障碍。MDCT 显示 13 名患者（65%）存在血管异常。3 人存在右锁骨下动脉异常，2 人存在左锁骨下动脉扩张，还有人存在主动脉根部扩张、主动脉憩室和左侧椎动脉异常。静脉异常方面，3 人存在部分肺静脉异常回流，2 人存在永存左上腔静脉。本研究使用 MDCT 发现无症状特纳综合征患者中有 65%存在心血管异常。即使特纳综合征患者没有心脏症状，在向成人过渡期间也必须进行心脏的超声心动图或 MDCT 全面评估。
