Objective: Neonatal vein of Galen malformation complicated by severe cardiac failure is a rare disease. The purpose was to assess the outcome of this life-threatening malformation and identify hemodynamic prognostic factors.
Design: Retrospective study.
Patients: Twenty-four newborns with cardiac failure requiring mechanical ventilation were consecutively admitted from 1986 to 2000.
Interventions: Cardiovascular evaluation including echocardiogram was performed in all cases. Eighteen transarterial shunt occlusions with glue were applied by the same team of three physicians.
Measurements and results: Twelve babies survived and underwent one endovascular session at least (median age 20 days) with a mean 63 months follow-up. Embolization was not performed in 6 of the 12 nonsurvivors because of severe brain damage or profound hypotension Cardiogenic shock occurred in all nonsurvivors, but also in one long-term survivor (p<0.0001). Echocardiogram showed signs of right ventricular failure, most often in the dead babies (p=0.005). The pulmonary systemic arterial pressure ratio was significantly higher in the nonsurvivor group (p=0.031), and it decreased significantly after the first embolization only in patients who survived (p=0.01). Patent ductus arteriosus and a diastolic aortic reversed-flow were present in all nonsurvivors in contrast to 30% of the long-term survivors (p=0.003 ). There was no difference in the left ventricular contractility and mean cardiac output between the two groups.
Conclusions: The outcome of vein of Galen malformation complicated by severe cardiac failure requiring mechanical ventilation remains poor. Neonatal embolization seems to be beneficial only in babies without suprasystemic pulmonary hypertension.