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Vein of Galen malformations are the most common form of symptomatic cerebrovascular malformation in neonates and infants. 1 Typically, newborns and young infants with a symptomatic vein of Galen malformation present with intractable heart failure, which is the most common cause of death in such patients. 1 2 Although there have been isolated case reports of congenital heart disease associated with arteriovenous malformations of the cerebral circulation (table 1 ), there have been no comprehensive reviews of this topic. After treating a patient who had an unusual and potentially serious complication resulting from the coexistence of these conditions, we reviewed published reports and our own experience to assess the incidence and distribution of congenital heart disease in neonates and infants with vein of Galen malformations.
Congenital cardiovascular anomaly | n | Reference |
Sinus venosus atrial septal defect | 6 | Friedman3 Hernandez4 Gomez5 Present report |
Coarctation of the aorta/isthmic hypoplasia | 9 | Gomez5 Comstock6 Reif7 Jellinger8 Clement9 Carroll 10 Present report |
Partially anomalous pulmonary venous drainage (of the right upper and middle pulmonary veins) to the superior vena cava | 1 1-150 | Levine 11 |
Ventricular septal defect | 3 | Clement9 Present report |
Transposition of the great arteries | 1 | Hirano 12 |
Tetralogy of Fallot | 1 | Rubinstein 13 |
Atrioventricular septal defect | 1 | Present report |
Secundum atrial septal defect | 2 | Present report |
Unspecified 1-151 | 1 | Lasjaunias 14 |
Because two patients in our series had both a ventricular septal defect and a secundum or sinus venosus atrial septal defect, individual lesions listed do not total 23, which is the total number of patients in whom defects were present.
This lesion is almost always found with a sinus venosus atrial septal defect, though the authors reported that there was no interatrial communication in this case.
A neonate with a vein of Galen malformation and surgical congenital heart disease was noted to have undergone cardiac surgery and died, but the type of heart defect was not specified.
Patients and abnormalities
Between 1983 and 1996, 25 neonates and infants with congenital vein of Galen malformations underwent echocardiography at our institution before surgical or endovascular treatment, some of whom were included in previous reports. 15 16 At the time of echocardiography, patients ranged in age from two fetuses at 36 weeks'...