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目的探讨主动脉缩窄(CoA)或主动脉离断(IAA)合并心内畸形的诊断及手术方式选择。方法2003年1月至2010年3月济宁医学院附属医院手术治疗14例小儿CoA(9例)或IAA(5例)合并心内畸形[包括室间隔缺损(VSD)、房间隔缺损(ASD)、房室隔缺损(APSD)等畸形,但不包括单纯合并动脉导管未闭(PDA)]患者,其中男10例,女4例;年龄0.7～12.0(3.2±4.5)岁;体重5.5～25.5(10.2±5.5)kg。分期手术3例,经胸骨正中切口径路一期手术矫治11例。结果手术死亡2例,其中1例为分期手术,二次手术行VSD修补术后死于急性左心衰竭;1例术前诊断为VSD+PDA合并重度肺动脉高压,术前未发现IAA,术后死于急性肾功能衰竭。随访12例,随访时间6～84(32±22)个月,患者恢复良好。复查心脏超声心动图提示:胸主动脉轻度狭窄2例,继续随访观察。结论多层螺旋CT和核磁共振成像是目前诊断CoA和IAA的首选检查方法。经胸骨正中切口径路一期手术治疗CoA或IAA合并心内畸形婴幼儿,可获较好的显露及疗效。
Objective To investigate the diagnosis and surgical treatment of aortic constriction (CoA) or aortic disconnection (IAA) with intracardiac deformity. Methods From January 2003 to March 2010, 14 cases of pediatric CoA (9 cases) or IAA (5 cases) with intracardiac malformation (including ventricular septal defect (VSD), atrial septal defect (ASD) (APSD) and other deformities, but not including patients with simple combined patent ductus arteriosus (PDA), including 10 males and 4 females, with an age of 0.7 to 12.0 (3.2 ± 4.5) years and a body weight of 5.5 to 25.5 (10.2 ± 5.5) kg. Staging surgery in 3 cases, the middle of the sternotomy approach surgery in 11 cases. Results Two patients died of surgery, one was staged and the other was died of acute left heart failure after VSD repair. One patient had preoperative diagnosis of VSD + PDA with severe pulmonary hypertension without preoperative IAA. Died of acute renal failure. All patients were followed up for 12 months. The follow-up time ranged from 6 to 84 (32 ± 22) months. The patients recovered well. Review echocardiography tips: 2 cases of mild thoracic aortic stenosis, follow-up observation. Conclusion Multi-slice spiral CT and magnetic resonance imaging are the first choice for the diagnosis of CoA and IAA. Through the sternotomy incision approach for the treatment of CoA or IAA with intra-heart deformity in infants and young children, can be a better exposure and efficacy.