Gold, et al.,20 firmly established the clinical features of aneurysms of the vein of Galen in 1964. They divided the clinical presentation into three characteristic age groups: that of the neonate, that of the infant, and that of the older child or adult. In neonates, congestive heart failure typically appeared shortly after birth; this was frequently cyanotic in type, and usually refractory to digitalis therapy. The infants presented with hydrocephalus or seizures, and older children or adults complained of headaches and/or signs and symptoms of subarachnoid hemorrhage (SAH).
In 1959, Silverman, et al.,49 first recognized congestive heart failure as the result of an aneurysm of the vein of Galen in a neonate. In patients with a large arteriovenous communication such as an aneurysm of the vein of Galen, there is a tendency to increased cardiac output as well as an increase of venous return to the heart. This results in pulmonary hypertension and progressive and intractable heart failure.25 Furthermore, because of the low diastolic pressure as the result of a large arteriovenous communication, there is reduced coronary blood flow, since coronary flow occurs predominantly during diastole. This leads to myocardial ischemia.
In addition to the intractable heart failure in these neonates, the tremendous flow of blood through the fistula deprives the cerebrum of blood and leads to gross parenchymal damage to the brain.39
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