单心室姑息治疗中进展失败的早期预测：为严重先天性心脏病个体化治疗迈出的一步。
Early prediction of failure to progress in single ventricle palliation: A step toward personalizing care for severe congenital heart disease.
机构信息
Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, California; Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, California; Heart Institute, Children's Hospital Los Angeles, Los Angeles, California; Keck School of Medicine, University of Southern California, Los Angeles, California.
Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, California; Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, California; Heart Institute, Children's Hospital Los Angeles, Los Angeles, California; Keck School of Medicine, University of Southern California, Los Angeles, California.
出版信息
J Heart Lung Transplant. 2022 Sep;41(9):1268-1276. doi: 10.1016/j.healun.2022.06.002. Epub 2022 Jun 6.
BACKGROUND
Advances in surgical technique and medical surveillance have improved outcomes of single ventricle (SV) palliation, particularly during the first interstage period. However, there remains a considerable mortality risk beyond this period.
METHODS
Patients born between January 2004 and December 2011 who required SV palliation were retrospectively identified. Patients who survived stage 1 palliation, were discharged home, and then were evaluated for Glenn candidacy, and continued care at our institution were included. Perioperative echocardiographic, hemodynamic, and operative data were analyzed at each surgical stage. The primary outcome was death or need for transplant. Univariate and multivariate analysis was completed using Cox proportional-hazards modeling.
RESULTS
A total of 175 patients were included. Three patients died after pre-operative evaluation before Glenn. Glenn was completed in 168 patients, 16 died before Fontan. Fontan was completed in 149 patients; 117 were alive without need for transplant, 17 died post-Fontan, and 1 required transplantation. Twenty-one patients were lost to follow-up throughout the study period and were censored at time of last follow-up. Pre-Glenn moderate or severe atrioventricular valve regurgitation (AVVR) was an independent risk factor for death/transplant (HR 2.41; p-value .026). Pre-Glenn moderate ventricular dysfunction was also an independent risk factor (HR 5.29; p-value .012). Other risk factors included right ventricular (RV) dominant morphology and perinatal acidosis.
CONCLUSIONS
Despite advances in SV palliation, a subset of these children remains at increased risk for poor outcomes. Early risk factors include RV dominant morphology and perinatal acidosis. Patients with substantial AVVR or ventricular dysfunction before Glenn palliation are also at significantly higher risk for death or requirement of transplantation later in childhood.
背景
手术技术和医疗监测的进步改善了单心室（SV）姑息治疗的结果，尤其是在第一中期阶段。然而，在此期间之后，仍然存在相当大的死亡风险。
方法
回顾性确定了 2004 年 1 月至 2011 年 12 月期间需要 SV 姑息治疗的患者。接受第一阶段姑息治疗后存活、出院并接受 Glenn 候选评估、继续在我院接受治疗的患者包括在内。在每个手术阶段分析围手术期超声心动图、血流动力学和手术数据。主要结局为死亡或需要移植。使用 Cox 比例风险模型进行单变量和多变量分析。
结果
共纳入 175 例患者。3 例患者在 Glenn 术前评估后死亡。168 例患者完成了 Glenn 手术，16 例在 Fontan 前死亡。Fontan 手术完成于 149 例患者，117 例无移植存活，17 例 Fontan 后死亡，1 例需要移植。21 例患者在整个研究期间失访，最后随访时被删失。术前 Glenn 中度或重度房室瓣反流（AVVR）是死亡/移植的独立危险因素（HR 2.41；p 值.026）。术前 Glenn 中度心室功能障碍也是独立危险因素（HR 5.29；p 值.012）。其他危险因素包括右心室（RV）优势形态和围产期酸中毒。
结论
尽管 SV 姑息治疗取得了进展，但其中一部分儿童的预后仍存在较高风险。早期危险因素包括 RV 优势形态和围产期酸中毒。在 Glenn 姑息治疗前有大量 AVVR 或心室功能障碍的患者在儿童后期死亡或需要移植的风险也显著增加。
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