High Output Cardiovascular Physiology and Outcomes in Fetal Diagnosis of Vein of Galen Malformation.
Simone Jhaveri, Alejandro Berenstein, Shubhika Srivastava, Tomoyoshi Shigematsu, Miwa K. Geiger.
Pediatric Cardiology (2021) 42:1416–1424
Take Home Points:
- Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications.
- Patients with VGAM develop superior vena cava dilation, reversal of diastolic flow in the transverse aortic arch, right ventricular (RV) and left ventricular (LV) dysfunction.
- RV dysfunction, more than mild tricuspid regurgitation and elevated combined cardiac index (CCI) are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss.
Commentary from Dr. Manoj Gupta (New York City, NY, USA), chief section editor of Pediatric & Fetal Cardiology Journal Watch.
Vein of Galen aneurysmal malformations (VGAM) are congenital cerebrovascular malformations that are characterized by multiple arteriovenous fistulas into choroidal veins that drain into a midline venous sac. The clinical presentation of patients with VGAM involves primarily cardiac and neurologic symptoms.
Neonates with large arteriovenous shunts from VGAM (choroidal type) typically present with high output cardiac failure, respiratory failure and pulmonary hypertension which almost universally results in death without intervention. Infants and older children may present with hydrocephalus, seizures, milestone regression, failure to thrive and rarely subarachnoid hemorrhage or other neurological impairments.
Interventional endovascular neurosurgical procedures, endovascular treatment of neonates with VGAM can be performed with a mortality of approximately 11%, good short-term neurologic outcomes and up to 80% cure in older children
Superior sinus venosus defects are associated with VGAM. All surviving patients have undergone successful neurosurgical interventions (n=4) of which two patients required procedures in the neonatal period prior to discharge. Of the non-survivors (n=5), one had received three neonatal interventions in the first week of life but died from intractable heart failure and sepsis. Three did not receive endovascular intervention and died within the first 48 h of life.
Comparison Between Survivors and Non-Survivors
When compared to survivors, non-survivors tended to more commonly have > mild TR (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was significantly higher in non-survivors as compared to survivors (672.7 vs. 530.2 ml/kg/min, p=0.016).
A higher CCI is seen in non-survivors as compared to survivors with VGAM. The majority of non-survivors had MRI findings not compatible with a good neurologic outcome. Measurement of ventricular functional parameters and cardiac outputs should be considered in the routine assessment of these patients.