Down综合征并发先天性心脏病的临床分析

Clinical analysis of Down syndrome with congenital heart diseases

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＋PDA2例，ASD＋VSD1例，ASD＋PDA＋VSD1例，VSD＋PDA4例，PDA十二叶主动脉瓣（BAV）1例，法洛四联症（TOF）2例，TOF＋ASD1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。

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 transtho- racic two-dimensional echoeardiography, 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 hyper- tension. 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 with- out pulmonary artery stenosis, about half are complicated with pulmonary artery hypertension.