Clinical And Echocardiographic Analysis Of Pediatric Patients With Turner Syndrome

Purpose: To study retrospectively the clinical data of pediatric patients with TS and perform echocardiography on them to analyze the cardiovascular structure and function.Method: During the period from 2015 to 2018,in the Department of Pediatrics,Tianjin Medical University General Hospital,16 pediatric patients with short stature and diagnosed as TS were collected and analyzed for all medical records.Echocardiography was performed in this group of pediatric patients with TS to observe cardiovascular structure and left ventricular function.The dimensions of the left ventricular end-diastolic diameter(LVIDd),left ventricular end-systolic diameter(LVIDs),interventricular septal end-diastolic thickness(IVSd),left ventricular posterior wall end-diastolic thickness(LVPWd),aortic valve annulus(AVA),aortic sinuses of Valsalva(ASV),sinotubular junction(STJ),ascending aorta(AAO)were measured.Left ventricular ejection fraction(LVEF),left ventricular mass(LVM),left ventricular mass index(LVMI)and Z scores for aortic diameter measurements at various sites were calculated.20 normal children with sex-and age-matched were selected as the normal control group.Echocardiography was performed at the same time,the data of the same parts were measured and calculated.According to LVEF and LVMI,the left ventricular function of pediatric patients with TS was analyzed.The Z scores of the aortic inner diameter of the TS group and the normal control group were compared.The difference was statistically significant at p< 0.05.Result: In the group of TS,there were 12 cases of pediatric patients with physical abnormalities,another 4 cases of pediatric patients without other physical abnormalities except short stature.Pelvic color Doppler ultrasound showed glandular dysplasia in 13 children,and 2 of them had abnormal levels of sex hormones.There were 10 patients with growth hormone deficiency,5 patients with thyroid abnormalities,and 15 patients with delayed bone age.Echocardiography revealed the presence of bicuspid aortic valve(BAV)in 1 case.The LVEF,LVMI and the Z scores of AVA,ASV and STJ in the TS group were not significantly different from those in the normal control group(p>0.05).The Z score of AAO in the TS group(0.46±0.79) was larger than that in the normal control group(-0.18±0.73),and the difference was statistically significant(p<0.05).Conclusion: There was a low proportion of 45,X chromosome karyotype in this group of pediatric patients of TS.These pediatric patients of TS with mild phenotype were characterized by short stature and may be combined with physical abnormalities and gonadal dysplasia.Cardiovascular structural abnormalities may occur in these patients in the early stage,but no left ventricular abnormality was found.Cardiovascular examination should be performed at regular intervals.The diameter of the ascending aorta in this group of pediatric patients of TS was broadened compared with normal children of sex-and age-matched.