Departments of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Cardiol Young. 2024 May;34(5):981-988. doi: 10.1017/S1047951123003542. Epub 2023 Nov 28.
Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation.
We sought to validate the score in an external, modern cohort.
This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation. Points were allocated per the NEONATE score (orwood type-Norwood/Blalock-Taussig shunt: 3, Hybrid: 12; extracorporeal membrane oxygenation post-op: 9, piates at discharge: 6, o Digoxin at discharge: 9, rch Obstruction on discharge echo: 9, ricuspid regurgitation ≥ moderate on discharge echo: 12; xtra oxygen plus ≥ moderate tricuspid regurgitation: 28). The composite primary endpoint was interstage mortality or heart transplant.
In total, 1026 patients met inclusion criteria; 61 (6%) met the primary outcome. Interstage mortality occurred in 44 (4.3%) patients at a median of 129 (IQR 62,195) days, and 17 (1.7%) were referred for heart transplant at a 167 (114,199) days of life. The median NEONATE score was 0(0,9) in those who survived to Stage 2 palliation compared to 9(0,15) in those who experienced interstage mortality or heart transplant (p < 0.001). Applying a NEONATE score cut-off of 17 points that separated patients into low- and high-risk groups in the learning cohort provided 91% specificity, negative predictive value of 95%, and overall accuracy of 87% (85.4-89.5%).
In a modern cohort of patients with single ventricle CHD and aortic arch hypoplasia, the NEONATE score remains useful at discharge post-Stage 1 palliation to predict freedom from interstage mortality or heart transplant.
源自国家儿科心脏病学质量改进协作注册中心，NEONATE 风险评分预测了单心室 CHD 和主动脉弓发育不良患者在 Stage 1 姑息治疗后出院回家时免于中期死亡或心脏移植的几率。
我们试图在一个外部的现代队列中验证该评分。
这是一项对 2016 年至 2020 年期间在国家儿科心脏病学质量改进协作二期注册中心登记的单心室 CHD 和主动脉弓发育不良患者的回顾性队列分析，这些患者在 Stage 1 姑息治疗后出院回家。根据 NEONATE 评分分配分数（伍德型-诺伍德/布莱洛克-塔斯克分流术：3 分，杂交术：12 分；术后体外膜氧合：9 分，出院时使用华法林：6 分，出院时使用地高辛：9 分，出院时心脏超声提示梗阻：9 分，出院时三尖瓣反流≥中度：12 分；体外氧合+≥中度三尖瓣反流：28 分）。主要复合终点为中期死亡或心脏移植。
共有 1026 例患者符合纳入标准；61 例（6%）发生了主要结局。44 例（4.3%）患者在中位时间 129（IQR 62，195）天发生中期死亡，17 例（1.7%）患者在 167（114，199）天的生命中被推荐进行心脏移植。与中期死亡或心脏移植的患者相比，存活至 Stage 2 姑息治疗的患者的 NEONATE 评分中位数为 0（0，9），而发生中期死亡或心脏移植的患者为 9（0，15）（p<0.001）。在学习队列中，应用 NEONATE 评分截断值 17 分将患者分为低风险组和高风险组，该评分具有 91%的特异性、95%的阴性预测值和 87%的总准确性（85.4-89.5%）。
在单心室 CHD 和主动脉弓发育不良患者的现代队列中，NEONATE 评分在 Stage 1 姑息治疗后出院时仍然有用，可预测中期死亡或心脏移植的风险。
