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Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings

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Abstract

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM.

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Abbreviations

ASD :

Atrial septal defect

AVM :

Arteriovenous malformation

CHD :

Congenital heart disease

CT :

Computerized tomography

LA :

Left atrium

MRI :

Magnetic resonance imaging

PAPVR :

Partial anomalous pulmonary venous return

RA :

Right atrium

RMPV :

Right middle pulmonary vein

RSVC :

Right superior vena cava

RUPV :

Right upper pulmonary vein

VGAM :

Vein of Galen aneurysmal malformation

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Authors and Affiliations

  1. Department of Pediatrics, Division of Cardiology, University of Texas Southwestern, 1935 Medical District Dr, Dallas, TX, 75235, USA

    Mehar Hoda, Matthew Lemler & Melinda Cory

Authors
  1. Mehar Hoda
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  2. Matthew Lemler
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  3. Melinda Cory
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Contributions

Mehar Hoda and Melinda Cory wrote the main manuscript text and Mehar Hoda prepared figures 1–4. Matthew Lemler critically reviewed the manuscript and provided edits to the main text and figures. All authors reviewed the manuscript.

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Correspondence to Mehar Hoda.

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Hoda, M., Lemler, M. & Cory, M. Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings. Pediatr Cardiol 44, 254–259 (2023). https://doi.org/10.1007/s00246-022-03057-x

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  • DOI: https://doi.org/10.1007/s00246-022-03057-x

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