中文摘要：

目的了解广东省中西南北部地区≤1岁婴儿先天性心脏病的发病状况。方法建立覆盖广东省的先天性心脏病防治监测网，其中监测单位34个。将在中西南北部地区监测单位首次诊断先天性心脏病且≤1岁的患儿列为监测对象。以小儿及胎儿超声心动图为诊断手段；统一专科培训、成立技术监测小组，以提高各网络单位的诊断水平。采用SPSS20．0软件对监测资料进行分析。结果2004年7月至2010年12月监测围产儿383281例，诊断≤1岁先天性心脏病患儿3263例。先天性心脏病总发病率为0．815％（3263／383281），其中男性发病率为0．868％（1799／207347），女性发病率为0．828％（1456／175843）；死产率高于活产率[10．627％（676／6361）比0．686％（2587／376920），P〈0．01]；珠江三角洲的先天性心脏病发病率高于非珠江三角洲[0．906％（2826／311823／）比0．611％（437／71458），P〈0．01]。活产儿先天性心脏病发病种类的前3位依次为室间隔缺损[占39．93％（1033／2587）]、动脉导管未闭[占29．84％（772／2587）]和继发性房间隔缺损[占13．76％（356／2587）]。结论广东省中西南北部地区先天性心脏病发病率处于全国中上水平，死产率较高，发病类型以室间隔缺损为主，珠江三角洲地区先天性心脏病发病率较高。

英文摘要：

Objective To summarize prevalence rate and region distribution of congenital heart disease （CHD） in 12-month-old and younger infants among four regions of Guangdong province, China. Methods Data from Guangdong CHD monitoring network including 34 monitoring units covering different geographic regions were analyzed. Professional training on screening and diagnosing CHD was provided to each work group member to improve the diagnosis level. CHD infants under or aged 12 months detected in the monitoring unit were included. CHD was diagnosed by fetus and infants echocardiography. Results From July 2004 to December 2010, 383 281 perinatal were registered and 3263 cases of CHD were detected in the 34 member units of Guangdong CHD monitoring network [ total prevalence rate of CHD： 0. 851% （3263/383 281 ） , male prevalence rate： 0. 868% （ 1799/207 347） , female prevalence rate：0. 828% （1456/175 843 ）]. Stillbirth CHD prevalence rate was significantly higher than livebirth CHD prevalence rate [ 10. 627% （676/6361） vs. 0. 686% （2587/376 920） ,P 〈 0. 01 ]. The total prevalence of CHD was significantly higher in Pearl River Delta region [ 0. 906% （2826/311 823 ） ] than in other regions [ 0. 611% （437/71 458 ）, P 〈 0. 01 J. Ventricular septal defect [ 39. 93% （ 1033/2587 ） in livebirth] was the most dominant CHD, followed by patent ductus arteriosus [ 29.84% （772/2587）] and secundum atrial septal defect [ 13.76 % （ 356/2587 ） ]. Conclusions The present data indicate that the prevalence of CHD in Guangdong is at the medium-upper level of the country associated with high stillbirth rate. The dominant type of CHD is ventricular septal defect. CHD prevalence is higher in the Pearl River Delta region than in other regions.