Challenging Case: Heart failure or Vein of Galen?

Vein of Galen preoperative imageryThe patient is a four-month-old boy from Haiti with a history of low weight gain, cachexia, congestive heart failure and pulmonary hypertension, but with no discernable cause. After first presenting to a hospital in Haiti, he was treated with oxygen concentrators and sildenafil to maintain his oxygen saturation levels, but could not gain weight.

After two months, the patient was seen by a cardiologist in Haiti, who identified a vein of Galen malformation by cranial ultrasound. This rare arteriovenous malformation is characterized by the presence of large, dilated blood vessels with abnormally high flow within the brain, sapping blood supply from the rest of the body and increasing stress on the heart, causing high-output congestive heart failure.

At age 10 months, the patient was transported from Haiti to Boston, where his care required a multidisciplinary team including cardiology, cardiac anesthesia, interventional neuroradiology and feeding and nutrition. After cardiologists in Boston Children’s Hospital’s Heart Center stabilized his heart, an interventional neuroradiologist with the hospital’s Vascular Anomalies Center and Cerebrovascular Surgery and Interventions Center conducted two rounds of embolization to close off most of the flow through the malformation.

Vein of Galen postembolization imageryWithin two weeks after the second embolization, the patient was moved out of intensive care and weaned off of supplemental oxygen shortly thereafter. He began to eat solid food and quickly gained appropriate weight.

At the time of discharge—less than one month after arriving in Boston—the patient was no longer in heart failure but continued to experience pulmonary hypertension.

He has returned to Haiti, where he continues to be monitored by Boston-based and Haitian doctors for pulmonary hypertension and any long-term sequelae stemming the now-treated malformation.