Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Division of Pediatric Cardiology, Department of Pediatrics, University of Mississippi Medical Center, 2500 N State Street, Jackson, MS, 39216, USA.
Pediatr Cardiol. 2022 Oct;43(7):1645-1652. doi: 10.1007/s00246-022-02895-z. Epub 2022 May 30.
Surgical options for coarctation of aorta (CoA) with atrioventricular septal defect (AVSD) include single-stage repair vs. staged approach with neonatal CoA repair and delayed AVSD repair. The durability of left atrioventricular valve (LAVV) function after neonatal repair is questioned, and the optimal approach remains controversial. Eighteen CoA-AVSD patients who underwent single-stage repair 2005-2015 by a single surgeon were retrospectively analyzed. Fifteen patients had complete and three had partial AVSD. Birth weight was 3.19 kg (2.17-4.08). Age at surgery was 16 days (6-127). One- and ten-year survival were 80% and 69%. Freedom from reintervention was 60% and 40% at one and ten-year respectively. Reinterventions included relief of left ventricular outflow tract obstruction (LVOTO) (n = 4), repair of cleft LAVV (n = 3), and LAVV and aortic valve replacement (n = 1). Freedom from LAVV reintervention was 85.6% and 66% at 1 and 10 years respectively. There were four deaths: two post-operative and two following hospital discharge. Mortality was due to sepsis in three patients, and heart failure related to LVOTO and LAVV insufficiency in one. At 68-month (0.6-144) follow-up the majority had mild or less LAVV regurgitation, and all had normal LV dimension and systolic function. There was no recurrent arch obstruction. Single-stage surgical repair of CoA-AVSD is feasible and reasonable. Survival and freedom from reintervention in our cohort approximate those outcomes of two-stage repair with durable left AV valve function and no recurrent arch obstruction. These patients are frequently syndromic and demonstrate mortality risk from non-cardiac causes. Consideration of a single-staged approach is warranted for appropriate patients with CoA-AVSD.
主动脉缩窄（CoA）伴房室间隔缺损（AVSD）的手术选择包括一期修复与分期修复，后者包括新生儿期 CoA 修复和延迟 AVSD 修复。新生儿期修复后左房室瓣（LAVV）功能的耐久性受到质疑，最佳方法仍存在争议。回顾性分析了 2005 年至 2015 年期间由一位外科医生行一期修复的 18 例 CoA-AVSD 患者。15 例患者完全性 AVSD，3 例部分性 AVSD。出生体重 3.19kg（2.17-4.08）。手术年龄 16 天（6-127）。1 年和 10 年生存率分别为 80%和 69%。1 年和 10 年无再次干预的生存率分别为 60%和 40%。再次干预包括解除左心室流出道梗阻（LVOTO）（n=4）、修复 LAVV 裂缺（n=3）和 LAVV 及主动脉瓣置换（n=1）。1 年和 10 年 LAVV 再次干预的无失败率分别为 85.6%和 66%。有 4 例死亡：术后 2 例，出院后 2 例。3 例死亡与败血症有关，1 例与 LVOTO 和 LAVV 功能不全引起的心衰有关。68 个月（0.6-144）随访时，大多数患者 LAVV 反流轻度或更轻，所有患者左心室大小和收缩功能正常。无再发弓部梗阻。CoA-AVSD 的一期手术修复是可行和合理的。我们的队列的生存率和无再次干预的生存率与分期修复相当，具有持久的左房室瓣功能和无再发弓部梗阻。这些患者经常为综合征患者，有非心脏原因导致的死亡风险。对于适当的 CoA-AVSD 患者，考虑行一期修复是合理的。
