Obara-Moszynska Monika, Rajewska-Tabor Justyna, Rozmiarek Szymon, Karmelita-Katulska Katarzyna, Kociemba Anna, Rabska-Pietrzak Barbara, Janus Magdalena, Siniawski Andrzej, Mrozinski Bartlomiej, Graczyk-Szuster Agnieszka, Niedziela Marek, Pyda Malgorzata
Department of Pediatric Endocrinology and Rheumatology, Poznan University of Medical Sciences, Poznan, Poland.
Cardiac Magnetic Resonance Unit, First Department of Cardiology, Poznan University of Medical Sciences, Poznan, Poland.
Front Endocrinol (Lausanne). 2018 Oct 16;9:609. doi: 10.3389/fendo.2018.00609. eCollection 2018.
Cardiovascular defects occur in 50% of patients with Turner syndrome (TS). The aim of the study was to estimate the usefulness of cardiac magnetic resonance imaging (CMR) and magnetic resonance angiography (angio-MR) as diagnostics in children and adolescents with TS. Forty-one females with TS, aged 13.9 ± 2.2 years, were studied. CMR was performed in 39 patients and angio-MR in 36. Echocardiography was performed in all patients. The most frequent anomalies diagnosed on CMR and angio-MR were as follows: elongation of the ascending aorta (AA) and aortic arch, present in 16 patients (45.7%), a bicuspid aortic valve (BAV), present in 16 patients (41.0%), and partial anomalous pulmonary venous return (PAPVR), present in six patients (17.1%). Aortic dilatation (-score > 2) was mostly seen at the sinotubular junction (STJ) (15 patients; 42.8%), the AA (15 patients; 42.8%), the thoracoabdominal aorta at the level of a diaphragm (15 patients; 42.8%), and the transverse segment (14 patients; 40.0%). An aortic size index (ASI) above 2.0 cm/m was present in six patients (17.1%) and above 2.5 cm/m in three patients (8.6%). The left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), and stroke volume (SV) were diminished (-score < -2) in 10 (25.6%), 9 (23.1%), and 8 patients (20.5%), respectively. A webbed neck was correlated with the presence of vascular anomalies ( = 0.006). The age and body mass index (BMI) were correlated with the diameter of the aorta. Patients with BAV had a greater aortic diameter at the ascending aorta (AA) segment ( = 0.026) than other patients. ASI was correlated with aortic diameter and descending aortic diameter (AD/DD) ratio ( = 0.002; = 0.49). There was a significant correlation between the right ventricular ( = 0.002, = 0.46) and aortic diameters at the STJ segment ( = 0.0047, = 0.48), as measured by echocardiography and CMR. Magnetic resonance can identify cardiovascular anomalies, dilatation of the aorta, pericardial fluid, and functional impairment of the ventricles not detected by echocardiography. BMI, age, BAV, and elongation of the AA influence aortic dilatation. The ASI and AD/DD ratio are important markers of aortic dilatation. The performed diagnostics did not indicate a negative influence of GH treatment on the cardiovascular system.
50%的特纳综合征（TS）患者会出现心血管缺陷。本研究的目的是评估心脏磁共振成像（CMR）和磁共振血管造影（血管造影-MR）在TS儿童和青少年诊断中的作用。对41名年龄为13.9±2.2岁的TS女性进行了研究。39例患者进行了CMR检查，36例进行了血管造影-MR检查。所有患者均进行了超声心动图检查。CMR和血管造影-MR诊断出的最常见异常如下：升主动脉（AA）和主动脉弓延长，16例患者（45.7%）出现；二叶式主动脉瓣（BAV），16例患者（41.0%）出现；部分肺静脉异位回流（PAPVR），6例患者（17.1%）出现。主动脉扩张（-评分>2）多见于窦管交界（STJ）（15例患者；42.8%）、AA（15例患者；42.8%）、膈肌水平的胸腹主动脉（15例患者；42.8%）和横段（14例患者；40.0%）。6例患者（17.1%）的主动脉尺寸指数（ASI）高于2.0 cm/m，3例患者（8.6%）高于2.5 cm/m。左心室舒张末期容积（EDV）、收缩末期容积（ESV）和每搏输出量（SV）分别在10例（25.6%）、9例（23.1%）和8例患者（20.5%）中减小（-评分<-2）。蹼颈与血管异常的存在相关（P=0.006）。年龄和体重指数（BMI）与主动脉直径相关。BAV患者升主动脉（AA）段的主动脉直径大于其他患者（P=0.026）。ASI与主动脉直径和降主动脉直径（AD/DD）比值相关（P=0.002；P=0.49）。通过超声心动图和CMR测量，STJ段的右心室直径（P=0.002，P=0.46）与主动脉直径之间存在显著相关性（P=0.0047，P=0.48）。磁共振能够识别超声心动图未检测到的心血管异常、主动脉扩张、心包积液和心室功能损害。BMI、年龄、BAV和AA延长会影响主动脉扩张。ASI和AD/DD比值是主动脉扩张的重要标志物。所进行的诊断未表明生长激素治疗对心血管系统有负面影响。
