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.

中文翻译：

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单心室姑息治疗失败的早期预测：向严重先天性心脏病个性化护理迈出的一步

背景

手术技术和医学监测的进步改善了单心室 (SV)姑息治疗的结果，特别是在第一个阶段间期。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 月出生的需要 SV 姑息治疗的患者。在 1 期姑息治疗中幸存下来的患者出院回家，然后接受了格伦候选资格评估，并包括在我们机构的继续护理。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。主要结果是死亡或需要移植。使用 Cox 比例风险模型完成单变量和多变量分析。

结果

共纳入 175 名患者。三名患者在格伦之前的术前评估后死亡。Glenn 完成了 168 名患者，其中 16 名在 Fontan 之前死亡。149 名患者完成了 Fontan；117 人在不需要移植的情况下存活，17 人在 Fontan 后死亡，1 人需要移植。21 名患者在整个研究期间失访，并在最后一次随访时被审查。Pre-Glenn 中度或重度房室瓣关闭不全 (AVVR) 是死亡/移植的独立危险因素（HR 2.41；p值 0.026）。Pre-Glenn 中度心室功能障碍也是一个独立的危险因素（HR 5.29；p值 0.012）。其他危险因素包括右心室 (RV) 主导形态和围产期酸中毒。

结论

尽管 SV 姑息治疗取得了进展，但这些儿童中的一部分仍然面临更高的不良结局风险。早期危险因素包括 RV 显性形态和围产期酸中毒。Glenn 姑息治疗前存在严重 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着增加。