【摘要】 目的分析先天性右位心病理特征,探讨其诊断及外科治疗。方法总结32例右位心临床资料,对其主要的合并畸形、诊断方法及其中16例外科治疗效果进行比较和分析。结果13例镜面右位心中8例存在大动脉转位(TGA)、单心室、动脉共干等复杂畸形;右旋心以生理矫正型TGA多见(11/17),且多数伴有室间隔缺损和肺动脉狭窄(8/11);2例孤立右位心则为TGA和完全型房室管畸形并存。主要根据X线检查(胸腹平片、肺门断层片、高千伏胸片、超高速CT)、超声心动图、心导管检查明确诊断。手术方法主要有:Fontan类手术8例,解剖矫治术7例,剖胸探查1例。存活13例病人中8例为复杂畸形,5例行改良Fontan术或全腔肺动脉连接术。结论镜面右位心也可合并复杂心血管畸形,右旋心、孤立右位心的合并畸形有一定规律;X线检查对右位心的诊断、分型有重要帮助,而超声心动图、超高速CT则是明确心内畸形和手术条件的主要手段;Fontan类手术(改良Fontan或全腔肺动脉连接术)是治疗复杂先天性右位心较有前途的方法,但应严格把握手术指征。更多还原

【Abstract】 Objective To understand the pathological features of congenital dextrocardia and explore its diagnosis and surgical treatment. Method The clinical data from 32 cases of congenital dextrocardia were collected to understand the major cardiac anomalies and evaluate the diagnostic approaches of this disease. Analysis of the effect of surgical treatment was also conducted in 16 among the 32 cases. Results Complex anomalies such as transposition of great artery (TGA), single ventricle (SV), common trunk (CMT) formation for the arteries were found in 8 out of 13 mirror-image dextrocardia cases, most of the dextroversion (DV) accompanied by physiological correction of the TGA (C-TGA, 11/17) which often involved ventricle septal defect (VSD) and pulmonary stenosis (PS, 8/11). Another 2 cases diagnosed as isolated dextrocardia (IDC) also suffered TGA combined with complete atrioventricular canal (CAVC) defect. Definite diagnoses of dextrocardia (DC) were derived from X-ray examination, including thoracic-abdominal plain X-ray film, hilar computed tomography, high kV chest plain film or ultrafast CT, echocardiogram and angiography. Fontan operation was performed in 8 cases and anatomical correction in 7 with surgical exploration in 1 case. Thirteen patients survived, among whom 8 had complex cardiac defect and 5 of them received modified Fontan operation or total cavopulmonary connection (TCPC). Conclusions Complex cardiac anomalies are not rare in mirror-image dextrocardia, and dextroversion and isolated dextrocardia were usually related to C-TGA+VSD+PS and TGA+CAVC respectively. X-ray examinations are of great significance in the diagnoses and classification of dextrocardia, and echocardiogram and ultrafast CT constitute the major modalities for clarifying intracardial defect and surgical indications. Fontan operations, including modified Fontan and total cavopulmonary connection, might be hopeful surgical treatment for complex congenital heart disease when indications are carefully evaluated.更多还原