VGAM induced high-flow congestive heart failure responsive to PGE1 infusion

Vein of Galen Aneurismal Malformation (VGAM), a rare congenital anomaly, can lead to congestive heart failure (CHF) and persistent pulmonary hypertension (PHT). Inotropic drugs, diuretics, mechanical ventilation or inhaled nitric oxide (iNO) are the main therapeutic strategies but often do not suffice to control the severe diastolic overload, thus other strategies must be sought. A term male infant was admitted on the second day of life with CHF. Echocardiography revealed normal cardiac anatomy but severe suprasystemic PHT and global volume overload. This led to the diagnosis of VGAM. CHF progressed rapidly to intractable cardiac failure refractory to diuretics, inotropics, oxygen supplementation and iNO. Prostaglandin E1 (PGE1) infusion was therefore started on the third day of life, while awaiting percutaneous occlusion of the fistula. With this treatment, cardiogenic shock could be reversed with rapid improvement of the existing lactic acidosis (lactate levels decreased from 17 to 4.5 mmol/L; base excess from -11.9 to -1.1). Unfortunately, during percutaneous embolisation sudden massive intracerebral hemorrhage occurred leading to death. Patients with refractory cardiac failure and suprasystemic pulmonary hypertension caused by VGAM might benefit of PGE1 infusion to ensure a decompressive right-to-left ductal shunting and thereby maintain an adequate systemic blood flow while awaiting treatment of the aneurismal arteries.