Background: Congenital heart defects are found in 50% of girls with Turner syndrome (TS). The evaluation of cardiovascular system is an important element in the diagnostic work-up of TS and is of particular significance of cardiologic monitoring, safety aspects of rGH treatment and any pregnancy planning.
Objective and hypotheses: Assess the cardiovascular system in TS girls with magnetic resonance imaging of the heart and aorta (CMR and angioMR).
Method: CMR and angioMR was performed in 30 children with TS. For the analysis of selected 26 girls. The mean age was 14.65 years. CMR was performed using 1,5T Magnetom Avanto machine. AngioMRI was performed with a gadoline contrast agent and type TWIST sequence. With a volumetric method using a CINE sequence the morphology and function of left and right ventricle were obtained. Phase contrast type sequences served for the assessment of a flow through the aorta and pulmonary truncus.
Results: Aortic diameters were measured at nine levels with maximum-intensity projection images. Regression analysis of diameters in relation to BSA demonstrated linear relationship between the cross-sectional aortic diameters and the square root of BSA (BSA0.5). Diameters were described regression function (−3.48+25.42*BSA0.5) mm for aortic sinus, (−1.52+20.39*BSA0.5) mm for sinotubular junction, (1.07+18.94*BSA0.5) mm for ascending aorta, (3.67+15.35*BSA0.5) mm for at the origin of brachiocephalic, (−4.62+21.26*BSA0.5) mm for first transverse segment, (−4.43+19.3*BSA0.5) mm for second transverse segment, (2.16+13.24*BSA0.5) mm for isthmic region, (6.21+9.08*BSA0.5) mm for descending aorta, (6.61+7.33*BSA0.5) mm for diaphragm. The results were compared to ranges developed by Kaiser et al. Comparison of the correlation coefficient of the regression of the study group and the regression function Kaiser revealed in the 7 to 9 aortic levels statistically significant difference. Aortic stenosis index >2.5 cm/m2 was in 1 patient.
Conclusion: CMR, particularly angioMRI, allows to detect vascular abnormalities of the aorta. The aorta dimensions are higher compared to the values in healthy population. AngioMRI is important tool for prognosis and planning further medical care.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology