Vein of Galen malformation

Case 1

MRI

MRI outlined the very significant Vein of Galen malformation leading to increased left to right shunt, leading to volume RV overload (and pulmonary vascular remodelling in fetal life). This patient presented with significant pulmonary hypertension - see ECHO below.

Echocardiography

Parasternal long axis view outlining some degree of RVOT dilation and Main Pulmonary artery dilation secondary to RV pressure and volume overload.

MPA dilation in PSAX view. Note the visible coronary arteries, likely easily visible from the increased pressure in the coronary sinus from the increased right atrial pressure due to the torrential SVC flow.

Views in Parasternal Short Axis view in B-Mode and Colour. There is trivial pulmonary insufficiency. The MPA is dilated.

Still view in colour of the left coronary that seems dilated.

Pulmonary insufficiency jet peak velocity at least of 30 mmHg, indicated some degree of increased diastolic pulmonary arterial pressure.

SVC flow by colour and PW Doppler. The SVC is dilated.

The right ventricle is dilated and hypertrophied. There is some trivial TR.

TRJ velocity (peak in systole) for a RV-RA gradient of 87 mmHg. RA pressure likely high for high venous return from SVC and dilated IVC/subhepatic veins in subcostal views. This indicates some increased systolic pulmonary arterial pressure.

Subcostal (subxyphoid) view showing the significant flow by the SVC and the right the left shunt by the ASD (/stretched PFO with redundant flap).

PW-Doppler in the SVC.

Short axis subcostal view showing the dilated SVC and IVC/subhepatic veins.

Subcostal long axis view of the SVC with colour. Inter-atrial right to left shunt.

Right to left shunt by the inter-atrial communication in the short axis subcostal view.

Sweep in short axis subcostal view indicating RV dilation and flattening of the septum systole and diastole.

Subcostal short axis sweep with colour.

Retrograde flow in the descending abdominal aorta.

Bidirectional shunt via the large ductus.

Case 2

Echocardiography

PLAX with significantly dilated RVOT

PLAX with RVOT dilation. Paradoxical movement of septum.

Dilated RVOT in the M-Mode in PLAX.

RVOT dilation and MPA dilation in the PSAX view.

Dilated MPA and mild pulmonary insufficiency by Colour.

PSAX outlining RV dilation, septal flattening in diastole and systole - suspicious for RV volume and pressure overload. Signs of some degree of LV compression ("pancaking").

PSAX with mild tricuspid regurgitation.

Apical 4 chamber view. There is RV dilation and some LV "pancaking". By colour, we can appreciate some mild TR and MR.

Mitral and Tricuspid insufficiency jet.

Significantly dilated SVC and innominate vein in suprasternal view.

Retrograde flow in the descending aorta.

Still-frame of dilated IVC.

SVC by colour.

Bidirectional but mostly right to left inter-atrial shunt.

Restrictive ductus - right to left in systole - indicating some degree of suprasystemic pulmonary pressure.

Head sonogram

Head sonography by 2D and colour ultrasound. One may appreciate the vein of Galen malformation with continuous flow and pocket of veins that are significantly dilated.

Created by Gabriel Altit - Neonatologist / Créé par Gabriel Altit (néonatalogiste) - © NeoCardioLab - 2020-2022 - Contact us / Contactez-nous