Vein of Galen Aneurysmal Malformation revealed by a severe heart failure and pulmonary hypertension in an infant

Abstract

Neonatal and pediatric heart failure is commonly caused by congenital heart diseases, especially by large left-to-right shunting. It is rarely related to a Vein of Galen Aneurysmal Malformation (VGAM). Diagnosis and management of these cerebrovascular malformations in infancy are still difficult and remain a medical challenge.
We report the case of a 2-month-old infant hospitalized for heart failure. In addition to symptoms relating to heart failure, the child presented in an agitated state with opisthotonic posture. Echocardiography showed dilated right heart cavities with pulmonary hypertension and no heart defect. Brain magnetic resonance imaging scanning confirmed the diagnosis of VGAM, showing multiple cerebral parenchyma damages. The baby was treated with diuretics and an angiotensin-converting-enzyme inhibitor leading to stabilization of her heart failure. Given the gravity of the neurological sequelae, no embolization procedure was planned for this child. She died 5 years later of acute heart failure.