Abstract
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 (Z-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/m2 was present in six patients (17.1%) and above 2.5 cm/m2 in three patients (8.6%). The left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), and stroke volume (SV) were diminished (Z-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 (p = 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 (p = 0.026) than other patients. ASI was correlated with aortic diameter and descending aortic diameter (AD/DD) ratio (p = 0.002; r = 0.49). There was a significant correlation between the right ventricular (p = 0.002, r = 0.46) and aortic diameters at the STJ segment (p = 0.0047, r = 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.
Highlights
Congenital and acquired cardiovascular disease occurs in almost 50% of patients with Turner syndrome (TS) [1] and are the major cause of death in TS, mostly due to dissection of the aorta [2]
The aim of the present study is to evaluate the usefulness of cardiac magnetic resonance imaging (CMR) and 3D dynamic magnetic resonance angiography in the diagnosis of anomalies of the aorta and other vessels and to establish risk factors for aortic dilatation in TS patients
The most frequent anomalies diagnosed on CMR and angio-MR were as follows: elongation of the AA and aortic arch in 16/35 (45.7%) patients, bicuspid aortic valve (BAV) in 16/39 patients (41.0%), partial anomalous pulmonary venous return (PAPVR) in 6/35 patients (17.1%), persistent left superior vena cava (PLSVC) in 4/35 patients (11.4%; Figure 4) and bovine arch in 3/35 patients (8.6%; Figure 5; Table 2)
Summary
Congenital and acquired cardiovascular disease occurs in almost 50% of patients with Turner syndrome (TS) [1] and are the major cause of death in TS, mostly due to dissection of the aorta [2]. The evaluation of the cardiovascular system is an important element of the diagnosis in TS. It is essential in terms of the safety aspects of recombinant growth hormone (rGH) treatment and pregnancy planning. Echocardiography is the most popular screening examination for cardiac anomalies in patients with TS. It can miss some cardiovascular anomalies [10, 11]. Assessment of the aortic arch and descending aorta on echocardiography can be limited by abnormalities of the chest wall and a poor acoustic window [5, 6, 12]. The aortic diameter is a risk factor for aortic dissection and can be monitored by cardiac magnetic resonance imaging (CMR)
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