Kuntz Michael, Staffa Steven, Valencia Eleonore, Kaza Aditya, Nasr Viviane G
Division of Pediatric Cardiac Anesthesia, Department of Anesthesiology Monroe Carell Jr. Children's Hospital at Vanderbilt Nashville TN USA.
Division of Cardiac Anesthesia, Department of Anesthesiology, Critical Care, and Pain Medicine Boston Children's Hospital, Harvard Medical School Boston MA USA.
J Am Heart Assoc. 2025 Jun 17;14(12):e039616. doi: 10.1161/JAHA.124.039616. Epub 2025 Jun 5.
Pathways for single-ventricle palliation include traditional neonatal surgical stage 1 palliation (SP), hybrid stage 1 palliation (HP; pulmonary artery flow restriction with ductal stenting), and delayed surgical stage 1 palliation (DSP), preceded by pulmonary artery flow restriction and prostaglandins. Findings from studies aiming to determine the optimal pathway are conflicting. We aimed to describe current pathway utilization and outcomes.
Using the Pediatric Health Information System between January 2016 and August 2023, we identified 1872 patients who underwent single-ventricle palliation (1573 SP, 123 DSP, and 176 HP).
Prematurity or low birth weight (<2.5 kg) were most common for DSP (52.9%) compared with SP (15.3%) and HP (36.4%) (<0.001). Comorbid conditions were most common for DSP. Case selection varied based on hospital volume; centers in the lowest volume quintile performed relatively more HP and DSP. In-hospital mortality and index hospitalization transplant were highest among HP (25.6%, 8.5%) compared with DSP (13.8%, 0.8%) and SP (11.3%, 0.9%) (<0.001). Hospital and intensive care unit length of stay were highest for DSP (both <0.001). Patients born prematurely or with low birth weight showed the highest in-hospital mortality for HP (35.9%) compared with DSP (16.9%) and SP (19.9%) (=0.012); postprocedure length of stay was longest for DSP (=0.026). The costs per day did not vary.
DSP was used for patients with a higher rate of prematurity, lower birth weight, and more noncardiac comorbid conditions. HP, however, was associated with higher in-hospital mortality and index hospitalization transplant. Further study to determine the potential benefit of DSP for high-risk patients is warranted.
单心室姑息治疗的路径包括传统的新生儿外科一期姑息治疗（SP）、杂交一期姑息治疗（HP；通过导管支架置入实现肺动脉血流限制）以及延迟外科一期姑息治疗（DSP），后者在肺动脉血流限制和使用前列腺素之后进行。旨在确定最佳治疗路径的研究结果相互矛盾。我们旨在描述当前治疗路径的使用情况和结果。
利用儿科健康信息系统，在2016年1月至2023年8月期间，我们确定了1872例接受单心室姑息治疗的患者（1573例SP、123例DSP和176例HP）。
与SP（15.3%）和HP（36.4%）相比，DSP患者中早产或低出生体重（<2.5 kg）最为常见（52.9%）（<0.001）。合并症在DSP患者中最为常见。病例选择因医院规模而异；规模最小的五分之一的中心进行的HP和DSP相对较多。与DSP（13.8%，0.8%）和SP（11.3%，0.9%）相比，HP患者的住院死亡率和指数住院移植率最高（25.6%，8.5%）（<0.001）。DSP患者的住院时间和重症监护病房住院时间最长（均<0.001）。与DSP（16.9%）和SP（19.9%）相比，早产或低出生体重的患者中HP的住院死亡率最高（35.9%）（=0.012）；DSP的术后住院时间最长（=0.026）。每日费用没有差异。
DSP用于早产率更高、出生体重更低且非心脏合并症更多的患者。然而，HP与更高的住院死亡率和指数住院移植率相关。有必要进一步研究以确定DSP对高危患者的潜在益处。
