无脾综合征患者合并复杂性先天性心脏病类型特点

发布时间：2018-11-24 15:30

【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。
[Abstract]:Objective: to investigate the anatomical characteristics and possible mechanism of the disease in patients with anisplenic syndrome and complicated intracardiac malformation. Methods: 47 patients (27 males and 20 females, aged 23 ~ 32 years) diagnosed by ultrasound without splenic syndrome were analyzed. The common intracardiac structural malformations and their frequency of occurrence in these syndromes were analyzed. Results: (1) Intracardiac malformations included the following 7 types: abnormal cardiac position in 16 cases (34.0%), extensive septal defects in 47 cases (100%), atrioventricular valve abnormalities in 42 cases (89.4%). There were 47 cases (100%) with abnormal origin of aorta, 46 cases (97.8%) with abnormal relationship of aorta, 45 cases (95.7%) with abnormal development of right ventricular outflow tract, pulmonary valve and pulmonary artery, 44 cases (91.5%) with abnormal systemic venous drainage. Pulmonary venous drainage was abnormal in 28 cases (59.6%). (2), cardiac malformations in these patients were usually accumulated at more than one site, including 1 case (2.1%) with 4 structural abnormalities, 5 cases (10.6%) with 5 kinds of structural abnormalities, 1 case (2.1%) with anomalous pulmonary venous drainage, 5 cases (10.6%) with abnormal pulmonary venous drainage. 13 cases (27.7%) were complicated with 6 structural abnormalities, 23 cases (48.9%) were complicated with 7 structural abnormalities, and 7 cases (14.9%) were complicated with 8 structural abnormalities. Conclusion: APS with intracardiac malformation is very complex, but it has obvious specificity. The presence of lateral dysfunction may be the underlying cause of a series of specific intracardiac deformities.
【作者单位】：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;首都医科大学附属北京儿童医院心脏超声科;
【分类号】：R541.1

【参考文献】