Flores Saul, Riley Christine M, Sassalos Peter, Smerling Jennifer, Cashen Katherine, Mastropietro Christopher W
Division of Critical Care and Cardiology, Department of Pediatrics, Texas Children's HospitalBaylor College of Medicine, 6651 Main Street, 14th Floor, Houston, TX, 77030, USA.
Children's National Health System, Washington, DC, USA.
Pediatr Cardiol. 2025 Mar;46(3):610-620. doi: 10.1007/s00246-024-03473-1. Epub 2024 Apr 3.
We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.
我们试图报告患有复杂型肺动脉起源异常左冠状动脉（ALCAPA）患儿的特征和结局，并将这些结局与孤立性ALCAPA患儿进行比较。我们对2009年1月至2018年3月期间在21个中心接受心脏手术的ALCAPA患儿进行了回顾性数据分析。使用描述性统计方法提供复杂型ALCAPA患者的特征和结局。使用Fisher精确检验比较复杂型ALCAPA和孤立性ALCAPA患者的结局。我们回顾了在21个中心接受ALCAPA手术修复的258例患者。我们确定了10例（3.9%）复杂型ALCAPA患者。初次心脏手术时的中位年龄为49天（范围：4天，12.8岁）。与ALCAPA相关的心脏病变包括左心发育不全综合征（n = 3）；弯刀综合征（n = 2）；室间隔缺损合并主动脉缩窄（n = 2）；室间隔缺损合并右肺动脉中断（1例）；法洛四联症合并二尖瓣闭锁（n = 1）；法洛四联症（n = 1）。1例患者在手术干预前诊断出ALCAPA；4例患者在初次心脏手术期间诊断出；3例患者在术后早期通过心导管检查诊断出；2例患者在初次手术修复后的儿童期后期诊断出。与孤立性ALCAPA患者相比，复杂型ALCAPA患者在ALCAPA修复后更有可能接受体外膜肺氧合（ECMO）治疗（50%对12%，p = 0.002）、接受心肺复苏（CPR）（30%对6%，p = 0.017）或发生手术死亡（50%对3%，p < 0.001）。复杂型ALCAPA并不常见。除1例患者外，所有复杂型ALCAPA患者术前均未被诊断，与孤立性ALCAPA患者相比，这些复杂型患者术后的发病率和死亡率明显更高。
