Department of Pediatrics Baylor College of Medicine Houston TX USA.
Section of Critical Care Medicine & Cardiology Texas Children's Hospital Houston TX USA.
J Am Heart Assoc. 2024 Jun 18;13(12):e032055. doi: 10.1161/JAHA.123.032055. Epub 2024 Jun 11.
We sought to measure frequency of achieving an optimal outcome after stage 1 palliation (S1P) for hypoplastic left heart syndrome and variants, determine factors associated with optimal outcomes, and compare outcomes after stage 2 palliation (S2P) using the National Pediatric Cardiology Quality Improvement Collaborative database (2008-2016).
This is a retrospective cohort study with optimal outcome defined a priori as meeting all of the following: (1) discharge after S1P in <19 days (top quartile), (2) no red flag or major event readmissions before S2P, and (3) performing S2P between 90 and 240 days of age. Optimal outcome was achieved in 256 of 2182 patients (11.7%). Frequency varied among centers from 0% to 25%. Factors independently associated with an optimal outcome after S1P were higher gestational age (odds ratio [OR], 1.1 per week [95% CI, 1.0-1.2]; =0.02); absence of a genetic syndrome (OR, 2.5 [95% CI, 1.2-5]; =0.02); not requiring a post-S1P catheterization (OR, 2.7 [95% CI, 1.5-4.8]; =0.01), intervention (OR, 1.5 [95% CI, 1.1-2]; =0.006), or a procedure (OR, 4.5 [95% CI, 2.8-7.1]; <0.001) before discharge; and not having a post-S1P complication (OR, 2.7 [95% CI, 1.9-3.7]; <0.001). Those with an optimal outcome after S1P had improved S2P outcomes including shorter length of stay, less ventilator days, shorter bypass time, and fewer postoperative complications.
Identifying patients at lowest risk for poor outcomes during the home interstage period could shift necessary resources to those at higher risk, alter S2P postoperative expectations, and improve quality of life for families at lower risk.
我们旨在衡量左心发育不全综合征和变体患者一期姑息治疗（S1P）后达到最佳结局的频率，确定与最佳结局相关的因素，并使用国家儿科心脏病学质量改进合作数据库（2008-2016 年）比较二期姑息治疗（S2P）后的结局。
这是一项回顾性队列研究，将最佳结局预先定义为满足以下所有标准：（1）S1P 后 19 天内（前四分之一）出院，（2）S2P 前无红旗或重大事件再入院，以及（3）在 90-240 天龄之间进行 S2P。在 2182 例患者中，256 例（11.7%）达到了最佳结局。各中心之间的频率从 0%到 25%不等。S1P 后达到最佳结局的独立相关因素为较高的胎龄（优势比[OR]，每增加一周增加 1.1[95%CI，1.0-1.2]；=0.02）；无遗传综合征（OR，2.5[95%CI，1.2-5]；=0.02）；无需 S1P 后行导管检查（OR，2.7[95%CI，1.5-4.8]；=0.01）、干预（OR，1.5[95%CI，1.1-2]；=0.006）或手术（OR，4.5[95%CI，2.8-7.1]；<0.001）在出院前；以及 S1P 后无并发症（OR，2.7[95%CI，1.9-3.7]；<0.001）。S1P 后达到最佳结局的患者，S2P 结局得到改善，包括住院时间更短、呼吸机天数更少、体外循环时间更短和术后并发症更少。
在家庭过渡期识别预后不良风险最低的患者，可以将必要的资源转移给风险更高的患者，改变 S2P 术后的预期，并提高低风险家庭的生活质量。
