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Vein of Galen Aneurysmal Malformation Associated with High Output Cardiac Failure in Three Neonates
Author(s) -
Malhotra Reenu Kapur,
Florez Luisa,
White Dollett,
Papasozomenos Sozos,
Covinsky Michael,
Bhattacharjee Meena,
Wang Min
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a76-b
Subject(s) - medicine , autopsy , heart failure , vein , cardiology , hydrocephalus , radiology
Vein of Galen Aneurysmal Malformation (VGAM) is a rare congenital vascular malformation resulting from communication between the cerebral arteries and vein of Galen. We report three autopsy cases, all prenatally diagnosed by ultrasound and/or fetal MRI with VGAM. All the three patients had high‐output cardiac failure associated with VGAM. Prenatal fetal echocardiogram on two patients showed cardiomegaly, ventricular dilatation, and primary pulmonary hypertension. The development of persistent primary pulmonary hypertension in these neonates is ominous and carries a poor prognosis. The cause of death in all the three patients was high‐output cardiac failure associated with VGAM. The autopsy findings corroborated with prenatal diagnosis of VGAM in all the patients. The other significant neuropathological findings were hydrocephalus, periventricular hemorrhage, periventricular leukomalacia, and microcalcification. A review of literature suggests that when a VGAM is large enough to be detected prenatally, the likelihood of perinatal cardiac failure increases dramatically. The overall neonatal mortality used to be about 90% before the advent of endovascular surgery. The prenatal documentation of brain atrophy and cardiac failure are associated with unfavorable outcomes in these infants.