Down综合征并发先天性心脏病的临床分析

目的:总结分析Down综合征(Down’s syndrome)并发的先天性心脏病畸形及血流动力学资料。方法:2008年7月~2012年10月,采用经胸二维超声心动图并彩色多普勒显像及右心导管/心血管造影检查方法,诊断36例并发先心病的Down综合征患者,本文通过36例临床资料分析,探讨Down综合征并发的先心病畸形及其血流动力学。结果:36例患者中室间隔缺损(VSD)10例,房室间隔缺损(AVSD)6例,动脉导管未闭(PDA)6例,房间隔缺损(ASD)2例,ASD+PDA 2例,ASD+VSD 1例,ASD+PDA+VSD 1例,VSD+PDA 4例,PDA+二叶主动脉瓣(BAV)1例,法洛四联症(TOF)2例,TOF+ASD 1例,18例有肺动脉高压者,其中5例为阻力型肺动脉高压。结论:①Down综合征并发的心血管畸形中,以VSD、AVSD和PDA最为常见,并常并发ASD、TOF。②在无肺动脉狭窄的患者中,约50%并发有肺动脉高压。

Clinical analysis of Down syndrome with congenital heart diseases

AIM:To analyze congenital heart deformity and hemodynamic data of Down syndrome. METHODS: Clinical data of Down’s syndrome with congenital heart disease and its hemodynamics were analyzed in 36 patients diagnosed with congenital heart disease patients with Down syndrome by transthoracic two-dimensional echocardiography, color Doppler flow imaging and right heart catheterization or angiocardiography from July 2008 to October 2012. RESULTS: Among the 36 cases, there were ten patients with VSD, six patients with AVSD, six patients with PDA, two patients with ASD, two cases with ASD+PDA, one case with ASD+VSD, one patient with ASD+PDA+VSD, four patients with VSD+PDA, one patient with PDA+BAV, two patients with TOF 2, and once patient with TOF+ASD. Eighteen patients had pulmonary hypertension including five cases of resistant pulmonary arterial hypertension. CONCLUSION: In cardiovascular malformations with Down syndrome, VSD (28%), AVSD (17%) and PDA (17%) are most common and are often associated with ASD or TOF. In patients without pulmonary artery stenosis, about half are complicated with pulmonary artery hypertension.