合并心内畸形的胸腹异位心脏的初步临床认识  

Primary Clinical Impression of Thoracoabdominal Ectopia Cordis With Intracardiac Abnormalities

作　　者：闫军[1,2] 萧明第[1,2] 刘迎龙[1,2] 孙寒松[1,2] 

机构地区：[1]中国医学科学院中国协和医科大学心血管病研究所心外科,北京市邮政编码100037 [2]中国医学科学院中国协和医科大学阜外心血管病医院心外科,北京市邮政编码100037

出　　处：《中国循环杂志》1996年第3期152-154,共3页Chinese Circulation Journal

摘　　要：目的:异位心脏是严重的心脏移位,极为罕见。我院外科收治两例合并心内畸形的胸腹异位心脏,现就临床特点结合文献资料总结如下。方法:两例患者诊断如下:①胸腹异位心脏,法乐四联症。②胸腹异位心脏,左室型单心室,共同心房,房耳并列,左位型大动脉异位,肺动脉瓣下肌性流出道肺动脉瓣及瓣上狭窄,完全性心内型肺静脉畸形引流,永存左上腔静脉,水平肝,脐突出。前一例家属对手术治疗有顾虑,未做。后一例接受了双侧上腔静脉分别与左右肺动脉端侧吻合(双侧双向Glenn术)和用牛心包片加强上腹前壁组织的手术。结果:后一例术后上腔静脉压一直高于2.67 kPa(20 mmHg)以上,有时3.33～3.87 kPa(25～29mmHg),可能因肺小血管发育不良,肺小血管阻力高,肺动脉高压,体循环难以维持,死亡。结论:胸腹异位心脏极为罕见,愈后差。合并心内畸形应行外科治疗。Objective: Ectopia cordis is a kind of severe and rare cardiac malposition. Two cases of the thoracoabdominal type with congenital intracardiac abnormalities were reported with review of relevent literature. Methods: Diagnosis of the two cases was as follows: one had thoracoabdominal ectopia cordis and tetralogy of Fallot, the other had thoracoabdominal ectopia cordis, left single ventricle, commom atrium, juxtaposition of two appendages, pulmonary stenosis, total anomalous pulmonary venous drainage, persistent left superior vena cava, horizontal liver, and omphalocele. The latter case underwent bidirectional Glenn shunt and prosthesis of the anterior upper abdominal wall with bovine pericardial patch. Results: The superior vena cava pressure of the latter case remained higher than 20 mmHg postoperatively, which might due to hypoplasia of the pulmgnary arterioles and pulmonary hypertension, died 8 days after operation. Conclusions: Thoracoabdominal ectopia cordis is rare with, poor prognosis, Surgical treatment is needed if associated with intracardiac abnormalities.

关 键 词：异位心脏 心内畸形 并发症 临床 

分 类 号：R541.1[医药卫生—心血管疾病]