Vein of Galen malformation with high output cardiac failure and premature endovascular therapy: a case report

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Abstract

Case Presentation: This case reports an-eight-months-old patient that was prenatally diagnosed with Vein of Galen Aneurysmal Malformation (VGAM), presenting cardiomegaly and severe anemia. At birth, progressed to severe pulmonary hypertension. At 11 days old, an early endovascular procedure was performed. Despite that, the patient progressed to high-output heart failure, relying on high doses of diuretic therapy, vasodilators, and oxygen therapy. A new CT angiography showed an aneurysmal venous structure with multiple dilated and tortuous supplying arteries. A new endovascular procedure was then performed. The child underwent cardiogenic shock in the immediate postoperative period. After a period of hospitalization, the patient was discharged at four months old, on room air, with an ascending weight curve, although still using diuretics to manage cardiac failure. The child has been reaching the neuropsychomotor development milestones corresponding to their chronological age.

Discussion: VGAM is a rare condition, corresponding to 1% of all cerebrovascular malformations, being the most frequent shunt in pediatrics. Untreated, they are 100% fatal. Heart failure, seen in most of these patients, is challenging to manage. Its total resolution is only possible with the correction of arteriovenous fistulas. The ideal time to perform the corrective procedure is a significant point of debate, the patient in question required two early interventions. More than 70% of the cases have a complete/near-complete resolution with only one embolization. However, subsequent approaches may be necessary. Up to 66% of patients will present complications with asymptomatic ventriculomegaly, but only 15% will present hydrocephalus with signs of intracranial hypertension. Previous studies have shown rates of adequate neuropsychomotor development in up to 60% of surviving cases. Recent studies, however, have shown that when prolonged neurological follow-up is performed up to the school stage, the prognosis may be more guarded. Even children with good neurological development will present weakness and deficits in memory and concentration.

Final Comments: Current endovascular intervention techniques have improved quality of life and prognosis. However, mortality rates can still reach up to 30%. It is essential for neurologists and pediatricians to know this pathology, its expected evolutionary course, and current evidence so they can identify delays and deficits in neurological development and thus provide early interventions.

Publication History

Article published online:
12 May 2025