الفهرس | Only 14 pages are availabe for public view |
Abstract Turner syndrome (TS) is caused by complete or partial monosomy of the X chromosome and has an overall prevalence of approximately 1 in 2500 live births of girls. It is characterized phenotypically by short stature and gonadal dysgenesis. The prevalence of cardiovascular abnormalities among patients with Turner syndrome varies in different series between 20% and 40%. It is higher in patients with monosomy X than in those with structural abnormalities of the X chromosome, and also higher in girls with a more pronounced clinical phenotype. Cardiovascular anomalies include aortic dilatation which may contribute to increased risk of aortic dissection. Thus, periodic cardiac magnetic resonance (CMR) imaging screening of persons with turner syndrome is beneficial. Although echocardiography was considered the mainstay of diagnosis, CMR can detect dilatation missed on echocardiography. Cardiac imaging, preferably magnetic resonance imaging, should be performed at diagnosis and repeated at 3 to 5 yr intervals to assess for congenital heart abnormalities and the emergence of aortic dilatation, a precursor to aortic dissection. Hypertension is a risk factor that should be aggressively treated |