无脾综合征患者合并复杂性先天性心脏病类型特点  

Clinical Features in Patients With Asplenia Syndrome Combining Complex Congenital Heart Disease

作　　者：孙妍[1,2] 王剑鹏[1] 李慧[1] 权欣[1] 张茗卉[1] 张丽[1] 徐宁[1] 王浩[1] 

机构地区：[1]中国医学科学院北京协和医学院国家心血管病中心阜外医院超声科,北京市100037 [2]首都医科大学附属北京儿童医院心脏超声科

出　　处：《中国循环杂志》2017年第7期672-675,共4页Chinese Circulation Journal

摘　　要：目的:统计分析无脾综合征合并复杂心内畸形患者心内畸形及其发生频次,探讨该疾病解剖学特征及可能机制。方法:分析47例超声诊断无脾综合征患者,其中男27例,女20例,年龄23天~32岁。统计该类综合征常见心内结构畸形及其发生频次。结果:(1)患者心内畸形主要包括以下7类:心脏位置异常16例(34.0%);大范围间隔组织缺损47例(100%);房室瓣异常42例(89.4%);主动脉起源异常47例(100%);大动脉相互关系异常46例(97.8%);右心室流出道、肺动脉瓣及肺动脉发育异常45例(95.7%);体静脉引流异常44例(91.5%);肺静脉引流异常28例(59.6%)。(2)该类患者心脏畸形常累计以上多部位,其中合并4种结构异常1例(2.1%);合并5种结构异常5例(10.6%);合并6种结构异常13例(27.7%);合并7种结构异常23例(48.9%);合并8种结构异常7例(14.9%)。结论:无脾综合征合并心内畸形虽然非常复杂,但具有明显的特异性。该病存在分侧功能障碍可能是造成一系列特异性心内畸形的根本原因。Objective： To statistically study the patients with asplenia syndrome combining complex congenital heart disease （CHD） for their common cardiac malformation, frequency of occurrence and to explore the anatomical features with possible mechanism. Methods： A total of 47 patients with asplenia syndrome were analyzed including 27 male and 20 female at the age from 23 days to 32 years. The common cardiac malformation and frequency of occurrence were statistically studied. Results： （1） The cardiac malformations were mainly with the following types： abnormal position of heart in 16 （34.0%） cases, a wide range of septal tissue defect in 47 （100%） cases, abnormal atrio-ventricular valve in 42 （89.4%） cases, abnormal aortic origin in 47 （100%） cases, abnormal position of 2 grate arteries in 46 （97.8%） cases, right ventricular outflow obstruction/pulmonary arterydysplasiain 45 （95.7%） cases, anomalous systemic venous drainage in 44 （91.5%） cases and anomalous pulmonary venous drainage in 28 （59.6%） cases. （2） The cardiac malformations were usually involved in several positions as 1 （2.1%） patient with 4 kinds of abnormal structures, 5 （10.6%） patients with 5 kinds of abnormal structures, 13 （27.7%） patients with 6 kinds of abnormal structures, 23 （48.9%） patients with 7 kinds of abnormal structures and 7 （14.9%） patients with 8 kinds of abnormal structures. Conclusion： Asplenia syndrome combining cardiac malformation has been complex while with specificity. Laterality dysfunction might be the primarycause for series malformations.

关 键 词：超声心动描记术 心脏缺损 先天性 无脾综合征 

分 类 号：R541[医药卫生—心血管疾病]