1临床资料患儿,男,7岁。因口唇青紫、胸闷伴有食欲不振急诊入院,入院查体:体质量14.3 kg,体温正常,口唇紫绀,呼吸急促,心率增快,双肺呼吸音清,未闻及干湿性罗音。下半段胸骨缺如,心脏可见搏动,下腹壁腹直肌缺如,可见一巨大脐疝,被覆皮肤,见图1A。超声心动图示:法乐四联症型右心室双出口、肺动脉瓣狭窄、房间隔缺损、三尖瓣关闭不全、永存左上腔静脉。诊断:法乐四联症型右心室双出口并发Cantrell五联症。手术采用胸部正中切口,术中探查见,患 1 clinical data children, male, 7 years old. Due to the lips bruising, chest tightness associated with loss of appetite emergency admission, admission examination: body weight 14.3 kg, normal body temperature, cyanotic lips, shortness of breath, rapid heart rate, lung breath sounds clear, unheard-of and wet and dry rales. The absence of the second half of the sternum, the heart can be seen pulsating, absent abdominal rectus abdominis, showing a huge umbilical hernia, covering the skin, see Figure 1A. Echocardiography: tetralogy of Fallot double right ventricle exports, pulmonary valve stenosis, atrial septal defect, tricuspid regurgitation, permanent left superior vena cava. Diagnosis: tetralogy of Fallot double right extubation Cantrell pentad. Surgical use of the median incision, intraoperative exploration see, suffering