Pulmonary hypertension associated with vein of Galen malformation. Fetal cardiac hemodynamic findings and physiological considerations

The management of newborns with vein of Galen aneurysmal malformation (VGAM) is clinically challenging for neonatologists and cardiologists. Hemodynamic profiles in four fetuses diagnosed with VGAM who subsequently developed neonatal cardiac failure and pulmonary hypertension were studied using two-dimensional and Doppler echocardiography. All four had an increased cardiothoracic ratio due to right ventricular dilatation on antenatal ultrasound. Doppler studies of the aortic isthmus were abnormal with retrograde flow starting in mid systole and throughout diastole. Left and right ventricular outputs were significantly increased. Net pulmonary flow was highly abnormal with a flow rate almost four times higher than normal. After a short period of clinical stability, all neonates developed cardiac failure and pulmonary hypertension. This article reviews VGAM pathophysiology and the potential relationship between pulmonary hypertension and VGAM, supporting early post-natal treatment of pulmonary hypertension and right ventricular failure.