镜面右位心的诊断和外科治疗——附11例临床分析

目的：分析镜面有位心病理解剖特点，探讨其临床诊断及外科治疗方法。方法：总结11例镜面右位心临床资料，对其主要的合并畸形、诊断方法和其中6例外科治疗的效果进行比较分析。结果：本组患者包括右心室双出口4例，单心室合并单心房2例，法乐四联征3例，完全性房室间隔缺损2例。合并畸形包括双上腔静脉3例，房间隔缺损3例，部分性肺静脉异位引流2例，三房心1例，动脉导管未闭1例。10例患者合并肺动脉狭窄或右室流出道狭窄，1例重度肺动脉高压。主要根据胸部X线片，超声心动图（UCG）及心导管明确诊断。行右心室双出口矫正术2例，法乐四联征根治术3例，右心室流出道疏通1例。4例存活，2例死亡。结论：（1）镜面右位心多存在复杂心内畸形。（2）UCG仍然是最基本有效的诊断方法，心导管检查是明确诊断的必要辅助手段。（3）外科治疗必须严格把握手术指征。

Diagnosis and surgical treatment of mirror-image dextrocardia:analysis of 11 cases

Objective: To analyze the pathological features and explore the diagnosis and surgical treatment of mirror-image dextrocardia. Methods: The clinical data of 11 cases of mirror-image dextrocardia were summarized, concerning with anomalies and diagnosis of UCG, and compared the effects of surgical treatment between 6 cases. Results: In this group, there were 4 cases with DORV (double outlet right ventricle), 2 with SV (single ventricle) and SA (single atrium), 3 with TOF (Tetrology of Fallot), 2 with CAVCD (complete atrial-ventricular canal defect), and 2 were combined with BISVC (bi-superior vena cava), 3 with ASD (atrial septal defect), 2 with PAPVD (partial anomalous pulmonary venous drainage), 1 with TA (tri-atriatum), and 1 with PDA (patent ductus arteriosus). 10 of them had PS (pulmonary stenosis) or RVOTO (right ventricular outlet tract obstruction). 1 patient was in pulmonary hypertention. Diagnosis was made mainly by chest X-ray, ultrasonic cardiogram and catheter. The surgical correction was performed for DORV on 2 cases, for TOF on 3 cases and enlarged right ventricular outflow tract on 1. 4 patients survived and 2 died. Conclusions: The patient with mirror-image dextrocardia is mostly to have complex anomalies in heart. UCG is still the most fundamental and effective method for diagnosis. Catheter is assistant to identify the diagnosis. It is possible for surgical treatment under strict condition.