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Isolated right superior vena cava draining into the left atrium in a child with vein of Galen aneurysmal malformation-case report

Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Ga...

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Published in:Journal of cardiothoracic surgery 2018-06, Vol.13 (1), p.66-66, Article 66
Main Authors: Elmahrouk, Ahmed F, Helal, Abdelmonem, Ismail, Mohamed F, Hamouda, Tamer, Mashali, Mohammed, Jamjoom, Ahmed A, Al-Ata, Jameel A
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description Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described. We describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully. The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.
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The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described. We describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully. 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subjects Age
Aneurysm
Atrium
Brain
Brain research
Cardiovascular system
Case Report
Case reports
Catheters
Congenital anomalies
Congenital defects
Congenital heart defects
Coronary artery disease
Cyanosis
Diagnosis
Echocardiography
Embolization
Emergency medical care
Endovascular embolization
Health aspects
Heart
Heart diseases
Medical imaging
Partial anomalous systemic venous drainage
Pediatrics
Septum
Surgeons
Surgery
Toddlers
Vein of Galen aneurysmal malformations
Veins & arteries
Venae cavae
Ventricle
Ventricles (cerebral)
title Isolated right superior vena cava draining into the left atrium in a child with vein of Galen aneurysmal malformation-case report
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