Sell J E, Jonas R A, Mayer J E, Blackstone E H, Kirklin J W, Castaneda A R
Boston Children's Hospital, MA 02115.
J Thorac Cardiovasc Surg. 1988 Dec;96(6):864-77.
Seventy-one patients with interrupted arch entered the Boston Children's Hospital between Jan. 1, 1974, and Jan. 1, 1987, of whom 63 underwent an operation. Type B was the most prevalent form of interrupted arch, and ventricular septal defect alone was the commonest coexisting cardiac anomaly. Among the 63, the 30-day and the 1-, 5-, and 10-year survival rates were 61%, 52%, 48%, and 47%, respectively. The mortality rate declined strikingly during the experience, and by multivariate analysis in patients with coexisting ventricular septal defect operated on in 1986, the probability of death within 2 weeks of repair was only 7%. Also, preoperative therapy became progressively more intense and more prolonged. The complication of left ventricular outflow tract obstruction developed in eight of the 33 patients undergoing repair of interrupted arch and of isolated ventricular septal defect. The time-related freedom from this complication was 97%, 78%, and 58% at 1 month, 1 year, and 3 years, respectively. Seven of the eight patients underwent a surgical procedure directed against the left ventricular outflow tract obstruction, and all have survived. Recurrent or persistent aortic arch obstruction became evident after repair in 15 patients and appeared more frequently and earlier after direct anastomosis than after tube graft repair. All patients had either reoperation or balloon dilation, but all were alive at follow-up. Most surviving patients are active and without symptoms. Inferences: An aggressive surgical program can result in survival and a good clinical state for at least 10 years after birth of over 40% of patients born with interrupted arch. Multiple anatomic bases account for the development of left ventricular outflow tract obstruction in about 50% of the patients undergoing repair of interrupted arch with coexisting ventricular septal defect. Repair by direct anastomosis combined with repair of the coexisting defect whenever possible is optimal therapy.
1974年1月1日至1987年1月1日期间，71例主动脉弓中断患者进入波士顿儿童医院，其中63例接受了手术。B型是主动脉弓中断最常见的类型，单独存在室间隔缺损是最常见的并存心脏异常。在这63例患者中，30天、1年、5年和10年生存率分别为61%、52%、48%和47%。在整个治疗过程中死亡率显著下降，通过对1986年接受手术的并存室间隔缺损患者进行多因素分析，修复后2周内死亡的概率仅为7%。此外，术前治疗逐渐变得更加强烈和持久。在33例接受主动脉弓中断修复和孤立室间隔缺损修复的患者中，有8例出现左心室流出道梗阻并发症。1个月、1年和3年时，无此并发症的时间相关概率分别为97%、78%和58%。8例患者中有7例接受了针对左心室流出道梗阻的手术，均存活下来。15例患者修复后出现复发性或持续性主动脉弓梗阻，直接吻合术后比人工血管移植修复后更频繁、更早出现。所有患者均接受了再次手术或球囊扩张，但随访时均存活。大多数存活患者活动自如且无症状。结论：积极的手术方案可使超过40%的主动脉弓中断患儿出生后至少10年存活且临床状态良好。多种解剖学基础导致约50%接受主动脉弓中断修复并存室间隔缺损患者发生左心室流出道梗阻。尽可能采用直接吻合联合并存缺损修复的方法是最佳治疗方案。
