Three newborn infants who had severe congestive heart failure caused by an arteriovenous malformation of the vein of Galen were evaluated by two-dimensional ultrasonography. Examination of the heart from standard echocardiographic planes showed right atrial and right ventricular dilatation. The superior vena cava, ascending aorta and the vessels to the head and neck were enlarged. Contrast two-dimensional echocardiography from a peripheral vein showed right-to-left atrial shunting and early superior vena cava recirculation. With the transducer positioned in the anterior fontanel or against the temporal bone, two-dimensional sector scans of the brain were obtained in the coronal, sagittal and transverse planes. A large echo-free space that represented an aneurysm of the vein of Galen was seen within the brain. During contrast injection into a peripheral vein, microcavitations that passed from right to left at the atrial level were seen filling the vein of Galen aneurysm. In two infants in whom beryllium wire was placed in the fistula to induce thrombosis, postoperative ultrasonography showed a dense mass of echoes arising from the wire and persistent left-to-right shunting through the fistula. In infants presenting in the first week of life with congestive heart failure and cyanosis of unknown etiology, two-dimensional ultrasonography provides a rapid, safe method for detecting intracranial arteriovenous malformation.
