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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。
Purpose: Ectopic heart is a severe heart shift, extremely rare. Surgical treatment of my courtyard two cases of intracardiac deformity of the chest and abdomen ectopic cardiac, clinical characteristics are now combined with the literature summarized as follows. Methods: Two patients diagnosed as follows: ① chest and abdomen ectopic heart, tetralogy of Fallot. ② chest and abdomen ectopic heart, left ventricular single ventricle, common atria, atrial and ear juxtaposition, left anterior descending aorta, pulmonary valve and pulmonary valve under the pulmonary valve stenosis, complete heart-type pulmonary venous anomalies drainage, Survival of the left superior vena cava, horizontal liver, umbilical prominent. The previous case of family members have concerns about surgery, did not do. The latter case underwent both sides of the superior vena cava and left and right pulmonary artery anastomosis (bilateral bidirectional Glenn surgery) and bovine pericardium to strengthen the anterior abdominal wall surgery. RESULTS: The posterior superior vena cava pressure was consistently above 2.67 kPa (20 mmHg), sometimes 3.33 to 3.87 kPa (25-29 mmHg), possibly due to poor development of pulmonary microvascular, high pulmonary vascular resistance, pulmonary hypertension, systemic circulation Difficult to maintain, death. Conclusion: Ectopic heart is extremely rare, the worse after. Merged heart deformities should be treated surgically.