内脏异位综合征的临床特征及死亡危险因素分析  

Analysis of clinical characteristics and death risk factors of patients with heterotaxy syndrome

作　　者：刘春洁[1] 李婷婷[1] 李奋[2] 徐让[3] LIU Chun-jie;LI Ting-ting;LI Fen;XU Rang(Department of Pediatric Cardiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China;Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China;Scientific Research Center, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China)

机构地区：[1]上海交通大学医学院附属新华医院小儿心血管科,上海200092 [2]上海交通大学医学院附属上海儿童医学中心心内科,上海200127 [3]上海交通大学医学院附属新华医院科研中心,上海200092

出　　处：《上海交通大学学报（医学版）》2016年第12期1772-1776,共5页Journal of Shanghai Jiao tong University:Medical Science

摘　　要：目的·分析内脏异位综合征患者死亡的危险因素。方法·收集56例明确诊断为内脏异位综合征患者的临床资料，分析其临床特征和死亡危险因素。结果·解剖分类：右侧异构47例，左侧异构9例，右侧异构比左侧异构合并较严重的心血管畸形。56例患者中死亡6例（10.7%），均为右侧异构，其中5例（83.3%）合并中重度房室瓣反流；Mann-Whitney秩和检验和χ2分析结果显示：死亡组与生存组患者中重度房室瓣反流和术后低血氧饱和度（≤80%）发生率的差异有统计学意义（P=0.0336，P=0.0417）；二分类Logistic回归分析显示，中重度房室瓣反流是内脏异位综合征患者死亡的危险因素[OR（95%CI）为11.666（1.254～108.557），P=0.0309]。结论·中重度房室瓣反流是内脏异位综合征患者死亡的重要危险因素。Objective · To analyze the risk factors for the death of patients with heterotaxy syndrome. Methods · The clinical data of 56 patients withclear diagnosis of heterotaxy syndrome were collected. Clinical characteristics and risk factors for death were analyzed. Results · 47 patients wereright isomerism and 9 patients were left isomerism according to anatomical classification. Patients with right isomerism had more severe cardiovascularmalformation than those with left isomerism. Of 56 patients, 6 (10.7%) with right isomerism died, in which 5 (83.3%) had moderate to severeatrioventricular valve regurgitation. Results of Mann-Whitney test and Chi-squared test showed that the differences in incidences of moderate to severeatrioventricular valve regurgitation and postoperative low blood oxygen saturation ( ≤ 80%) were statistically significant (P=0.033 6, P=0.041 7).Univariate Logistic regression analysis showed moderate to severe atrioventricular valve regurgitation was a risk factor for the death of patients withheterotaxy syndrome [OR (95% CI): 11.666 (1.254-108.557), P=0.030 9]. Conclusion · Moderate to severe atrioventricular valve regurgitation is animportant risk factor for the death of patients with heterotaxy syndrome.

关 键 词：内脏异位综合征 心血管畸形 危险因素 

分 类 号：R541.1[医药卫生—心血管疾病]