关键词: 特纳综合征, 心血管异常, 超声心动图

Abstract: Objective To analyse the associated cardiovascular abnormalities in patients with Turner's syndrome (TS). Methods Clinical features and cardiovascular abnormalities of 25 female patients with TS were analyzed according to karyotype. Results Congenital cardiovascular malformations were detected in 40% of TS patients, including bicuspid aortic valve, mitral/aortic lengthy or prolapse, coronary sinus dilation, cardiac chamber dilation, and noncompaction of the ventricular myocardium. The patients with monosomy X, more often than those with mosaicism, had a higher incidence of congenital cardiovascular malformations (40% vs 14%, p<0.01). Aortic size index, including root and assending aorta were significantly greater in TS than the healthy contro(27±5mm vs 18±2mm, 28±6mm vs 18±2mm, p<0.001). Conclusion Regular evaluation of congenital cardiovascular malformations and accompanied risk factors may help to predict the risk of cardiovascular death.

Key words: Turner's Syndrome, cardiovascular abnormalities, echocardiography