PM1005: Echocardiography Evaluation of Neonatal Vein of Galen Aneurysmal Malformation

Background: Neonates with vein of Galen aneurysmal malformation (VGAM) can present with significant high output heart failure and pulmonary hypertension. Echocardiogram is the primary imaging modality to evaluate the cardiac effects of VGAM. Some neonates need early neuro-intervention to control heart failure and prevent multi-organ failure, while others can be medically managed until 4-6 months of age when elective neuro-intervention can be performed. There is paucity of data regarding the role of echocardiography in determining severity, need for early intervention and outcomes of this rare malformation. The objective of our study is to describe and compare echocardiographic parameters in neonates with aggressive versus non-aggressive course due to VGAM. Our secondary objective is to compare echocardiographic parameters in patients who survived vs died of VGAM at their last follow up.

Methods: We reviewed the medical charts of all patients with the diagnosis of VGAM at Children’s Medical Center, Dallas after obtaining approval from internal review board between January 2007 and March 2022. We excluded patients in whom the diagnosis was made beyond 28 days of age and those who did not have an echocardiogram at our institution prior to their first intervention. We assessed echocardiographic parameters that have been previously reported to have an association with disease severity in patients with VGAM and compared them between patients who had aggressive versus non-aggressive disease. We define aggressive disease as the need for embolization within 7 days of life and/or death within 7 days of life. We also compared the parameters between patients who died vs survived.

Results: Of 31 patients with the diagnosis of VGAM, 19 patients met the inclusion and exclusion criteria. The median birth weight was 3.09 (IQR 2.58 – 3.36) kg with equal male and female distribution. The median age of the first post-natal echocardiogram was 2 (IQR 1 – 5.5) days. Ten patients were categorized as having aggressive disease whereas the remaining 9 had non-aggressive disease. Of the 19 patients, 9 (47.4%) died at median age of 5 (IQR 4-17) days. The median age of the remaining 10 patients alive at their last follow up was 465 (IQR 130 – 1613) days. There was no significant difference in the echocardiographic parameters between patients with aggressive and non-aggressive disease (Table 1) or in patients that survived vs died (Table 2).

Conclusion: In the current era, the mortality of neonatal VGAM remains high. Though there is interest in using echocardiographic parameters to determine need for early neuro-intervention, we were unable to identify a parameter that is predictive of severe disease. The data is limited by the small number of patients with this rare disease.