INTRODUCTION Vein of Galen aneurysmal malformation (VGAM) is a rare, congenital cerebrovascular malformation with high morbidity and mortality. Parameters to foresee clinical progression and allow individualized parent counseling are lacking. The aim of this study was to evaluate aortic steal measured by Doppler ultrasound as a prognostic parameter in these neonates. METHODS A retrospective monocentric analysis of cardiac ultrasound exams before embolization in neonates with VGAM was conducted. Percentage of aortic steal measured by time-averaged maximum velocity above and below the zero flow baseline by pulsed Doppler ultrasound at the preductal aortic isthmus was calculated. Association of aortic steal with parameters of acute organ dysfunction (Bicêtre neonatal evaluation score [BNES], neonatal multiple organ dysfunction score [NeoMODS]) and mortality and determination of correlation between aortic steal and cerebral damage on initial and follow-up cerebral magnetic resonance imaging (cMRI) were evaluated. RESULTS Twelve neonates were included, of which 3 died. Per 10 percentage point increase of aortic steal, BNES decreased by 1.64 (95% confidence interval [CI]: 1.28-2.0) points and the maximum observed NeoMODS increased by 1.25 (CI: 0.94-1.57) points. The odds for mortality increased by 2.3 (CI: 1.14-13.67) per 10 percentage point increase of aortic steal. There was a correlation between aortic steal and cerebral damage at baseline (white matter ρ [rho] = 0.34, gray matter ρ = 0.81) and follow-up (white matter ρ = 0.80, gray matter ρ = 0.72). CONCLUSION The degree of aortic steal in neonates with VGAM was highly associated with the severity of organ dysfunction, disease progression on cMRI, and mortality.

中文翻译：

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主动脉盗血与盖伦静脉畸形新生儿的急性器官功能障碍和短期结局相关。

引言 Galen 静脉动脉瘤畸形 (VGAM) 是一种罕见的先天性脑血管畸形，发病率和死亡率很高。缺乏预见临床进展和允许个性化家长咨询的参数。本研究的目的是评估多普勒超声测量的主动脉窃血作为这些新生儿的预后参数。方法 对 VGAM 新生儿栓塞前的心脏超声检查进行回顾性单中心分析。通过脉冲多普勒超声在导管前主动脉峡部测量高于和低于零流量基线的时间平均最大速度来计算主动脉窃血的百分比。主动脉盗血与急性器官功能障碍参数（Bicêtre 新生儿评估评分 [BNES]、新生儿多器官功能障碍评分 [NeoMODS]）和死亡率的关联，以及在初始和随访脑磁共振成像中确定主动脉盗血与脑损伤之间的相关性（cMRI）进行了评估。结果 纳入新生儿12例，其中3例死亡。主动脉盗血每增加 10 个百分点，BNES 就会减少 1.64（95% 置信区间 [CI]：1.28-2.0）点，观察到的最大 NeoMODS 增加 1.25（CI：0.94-1.57）点。主动脉盗血每增加 10 个百分点，死亡率就会增加 2.3 个百分点（CI：1.14-13.67）。主动脉窃血与基线（白质 ρ [rho] = 0.34，灰质 ρ = 0.81）和随访（白质 ρ = 0.80，灰质 ρ = 0.72）的脑损伤之间存在相关性。结论 VGAM 新生儿的主动脉盗血程度与器官功能障碍的严重程度、cMRI 上的疾病进展和死亡率高度相关。