先天性右位心的诊断及其合并畸形的外科治疗

目的分析先天性右位心病理特征,探讨其诊断及外科治疗。方法结合文献,总结2002-2010.6年收治的63例右位心病例资料,对其主要的合并畸形,诊断方法及其中9例外科治疗手术方法、效果进行总结。结果右位心常合并多种心内畸形,43例先天性镜面右位心中20例存在心内畸形,常见合并畸形为完全性大动脉转位,其次为室间隔缺损、肺动脉狭窄、房间隔缺损、单心室等;20例先天性右旋心中19例存在心内畸形,以大动脉转位、室间隔缺损多见。63例均依靠胸部X线检查、心电图及超声心动图明确诊断。手术9例术后诊断与超声诊断完全一致,均存活。其中5例合并简单畸形,通过手术得到解剖矫治,心功能1级,效果好;4例合并复杂畸形,3例行根治术后心功能1～2级,1例行姑息性手术,生活质量明显改善,效果满意。结论先天性镜面右位心合并心脏畸形的诊断需要结合辅助检查结果确诊,超声心动图可作为右位心诊断的首选或筛选方法,X线检查对右位心的诊断、分型有重要帮助。镜向右位心的心电图有特征性改变。右位心未合并心内畸形无需特殊处理;合并心内畸形应根据解剖和病理生理情况选择手术治疗。

The diagnosis and surgical treatment of congenital dextrocardia with malformations

【Objective】 To evaluate the pathological features of congenital dextrocardia,discuss its value in the diagnosis and surgical treatment.【Methods】 With relevant literature,We have analyzed clinical data of 63 cases patients with dextrocardia.Then summarize the major malformations,diagnostic program and surgical treatment programs and efficacy of 9 cases.【Results】 It is often associated with multiple cardiac malformations in dextrocardias.Among 43 patients diagnosed as mirror-image dextrocardia,20 patients were found to have concomitant cardiovascular malformations,the major anomalies were completed transposition of main arteries,followed by ventricular septal defect,pulmonary stenosis,atrial septal defect,single ventricle,etc;in 20 cases of dextroversion,19 patients were diagnosed as concomitant cardiovascular malformations,such as transposition of main arteries,ventricular septal defect.All the 63 cases were diagnosed clearly by the chest X-ray examination,electrocardiogram and echocardiography.The postoperative diagnosis of 9 patients received surgical treatment is fully consistent with the ultrasound diagnosis,and all the 9 is survived among them,5 patients with Simple deformity achieved good treatment after surgery with cardiac function the first degree.4 patients with complex malformations,three of them received radical surgery have the cardiac function degree 1-2,the last one take the palliative surgery achieved grdat treatment and improved the quality of life significantly.【Conclusion】 It must relay on supplementary examination to confirm diagnosis of the congenital mirror-image dextrocardia patients associated with cardiac malformations.Echocardiography can be used as preferred or screening method in the diagnose of dextrocardias,while,X-ray play an important role in the diagnosis and classification.Mirror-image dextrocardia has a characteristic ECG changes.Mirror-image dextrocardias,to those associated with no cardiac malformations required not special treatment,on the contrary,to those with cardiac malformations required surgical treatment according to their anatomy and pathophysiology.