Oosterhof Thomas, Azakie Anthony, Freedom Robert M, Williams William G, McCrindle Brian W
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Ann Thorac Surg. 2004 Nov;78(5):1696-702. doi: 10.1016/j.athoracsur.2004.05.035.
Interrupted aortic arch (IAA) continues to be associated with important mortality, both before and immediately after repair, with ongoing morbidity during follow-up. We sought to determine trends in presentation, management, outcomes and associated factors.
We reviewed all consecutive patients (n = 119) presenting from 1975 to 1999, and data were collected regarding demographics, anatomy, management and outcomes.
Significant trends over time for patients born in three consecutive periods (1975 to 1984, 1985 to 1993, and 1994 to 1999) demonstrated a smaller proportion of patients with presentation with circulatory collapse (65%, 51%, and 25%, respectively), greater use of prostaglandins (72%, 90%, 100%), fewer deaths without IAA repair (49%, 15%, 13%) and greater use of one-stage repair (68%, 75%, 100%). Independent risk factors for death without IAA repair (p < 0.001) included absence of ventricular septal defect, and the presence of noncardiac anomaly, complex cardiac anomaly, episode of acidosis and earlier birth cohort. Overall survival after repair was 50% at age 1 month, 35% at 1 year, and 34% at 5 years. Early and constant-hazard phases were noted, with incremental risk factors for early phase mortality being cyanosis at presentation, presence of truncus arteriosus or aortic stenosis, an episode of circulatory collapse before repair, earlier date of repair, and lower weight at repair. Greatest survival occurred in those patients with uncomplicated IAA who had repair since 1993 (5 year survival, 83%). Freedom from reintervention for arch obstruction was 60% at 5 years.
While improving, outcomes of IAA remain of concern, especially in patients with associated lesions.
主动脉弓中断（IAA）在修复前及修复后即刻仍与较高的死亡率相关，且随访期间存在持续的发病率。我们试图确定其临床表现、治疗、结局及相关因素的趋势。
我们回顾了1975年至1999年期间所有连续就诊的患者（n = 119），并收集了有关人口统计学、解剖结构、治疗及结局的数据。
连续三个时期（1975年至1984年、1985年至1993年、1994年至1999年）出生的患者随时间呈现出显著趋势，表现为出现循环衰竭的患者比例较小（分别为65%、51%和25%），前列腺素的使用更为广泛（72%、90%、100%），未进行IAA修复而死亡的患者较少（49%、15%、13%），且一期修复的使用更为广泛（68%、75%、100%）。未进行IAA修复而死亡的独立危险因素（p < 0.001）包括无室间隔缺损、存在非心脏异常、复杂心脏异常、酸中毒发作及较早的出生队列。修复后的总体生存率在1个月时为50%，1岁时为35%，5岁时为34%。观察到早期和恒定风险期，早期死亡的递增危险因素包括就诊时的青紫、存在动脉干或主动脉狭窄、修复前的循环衰竭发作、较早的修复日期及修复时较低的体重。1993年以来进行修复的无并发症IAA患者生存率最高（5年生存率，83%）。5年时免于因主动脉弓梗阻再次干预的比例为60%。
虽然情况有所改善，但IAA的结局仍令人担忧，尤其是在伴有相关病变的患者中。
