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Perinatal Pseudocoarctation

Echocardiographic Findings in Vein of Galen Malformation

Division of Maternal Fetal Medicine, Department of Obstetrics, Gynecology, and Reproductive Sciences (N.M.D., J.M.M.), and Division of Pediatric Cardiology, Department of Pediatrics (M.K.T., M.R.L.-H.), University of Texas Houston Medical School, Houston, Texas USA; and Memorial Hermann Children’s Hospital, Houston, Texas USA (M.R.L.-H.).

Address correspondence and reprint requests to M. Regina Lantin-Hermoso, MD, Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Houston Medical School, 6431 Fannin, MSB 3.126, Houston, TX 77025 USA. E-mail: m.r.lantin-hermoso{at}uth.tmc.edu

Objective. Vein of Galen aneurysmal malformations (VGAMs) are rare congenital malformations thought to develop during weeks 6 to 11 of fetal life. Although they represent less than 1% of all cerebral vascular malformations, they constitute up to 30% of all pediatric vascular malformations. Vein of Galen aneurysmal malformations cause high-output heart failure in the fetus and neonate secondary to the decreased resistance and high blood flow in the lesion. We describe 2 cases, 1 prenatal and 1 postnatal, in which unusual aortic Doppler flow patterns and substantial brachiocephalic vessel dilation contributed to the discovery of a VGAM. Methods. Echocardiographic findings associated with VGAM malformations in 2 cases are described. Results. Unusual Doppler flow patterns and substantial brachiocephalic vessel dilation were seen in both cases. Pseudocoarctation of the aorta was also noted in both cases. Conclusions. The echocardiographic findings in fetal and neonatal VGAM may include pseudocoarctation of the aorta. Abnormal fetal cardiac findings should raise the practitioner’s suspicion for cerebral malformations and vice versa.

Key Words: prenatal • postnatal • pseudocoarctation of aorta • vein of Galen aneurysmal malformation

Abbreviations: MRI, magnetic resonance imaging • PDA, patent ductus arteriosus • VGAM, vein of Galen aneurysmal malformation

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