上海交通大学学报（医学版）

上海交通大学学报（医学版）

• 论著（临床研究） • 上一篇    下一篇

内脏异位综合征的临床特征及死亡危险因素分析

刘春洁 1，李婷婷 1，李奋 2，徐让 3   

  1. 上海交通大学 医学院 1.附属新华医院小儿心血管科，上海 200092; 2.附属上海儿童医学中心心内科，上海 200127; 3.附属新华医院科研中心，上海 200092
  • 出版日期:2016-12-28 发布日期:2016-12-29
  • 通讯作者: 徐让，电子信箱：rang_xu@hotmail.com。
  • 作者简介:刘春洁（1992—)，女，硕士生；电子信箱：liucj92@163.com。

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

LIU Chun-jie1, LI Ting-ting1, LI Fen2, XU Rang3   

  1. 1. Department of Pediatric Cardiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China; 2. Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China; 3. Scientific Research Center, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China

  • Online:2016-12-28 Published:2016-12-29

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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=0.033 6，P=0.041 7）；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=0.030 9]。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。

关键词: 内脏异位综合征, 心血管畸形, 危险因素

Abstract:

Objective · To analyze the risk factors for the death of patients with heterotaxy syndrome. Methods · The clinical data of 56 patients with clear diagnosis of heterotaxy syndrome were collected. Clinical characteristics and risk factors for death were analyzed. Results · 47 patients were right isomerism and 9 patients were left isomerism according to anatomical classification. Patients with right isomerism had more severe cardiovascular malformation than those with left isomerism. Of 56 patients, 6 (10.7%) with right isomerism died, in which 5 (83.3%) had moderate to severe atrioventricular valve regurgitation. Results of Mann-Whitney test and Chi-squared test showed that the differences in incidences of moderate to severe atrioventricular 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 with heterotaxy syndrome [OR (95% CI): 11.666 (1.254-108.557), P=0.030 9]. Conclusion · Moderate to severe atrioventricular valve regurgitation is an important risk factor for the death of patients with heterotaxy syndrome.

Key words: heterotaxy syndrome, cardiovascular malformation, risk factors