Isolated right superior vena cava draining into the left atrium in a child with vein of Galen aneurysmal malformation\u2014case report

Ahmed F Elmahrouk,Mohamed F Ismail,Ahmed A Jamjoom,Abdelmonem Helal,Jameel A Al-Ata,Tamer Hamouda,Mohammed Mashali

doi:10.1186/s13019-018-0758-x

Abstract

BackgroundIsolated 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.Case presentationWe 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.ConclusionsThe 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.

Highlights

Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition
The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the left atrium (LA), causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs
The high-flow, low-resistance arteriovenous connection in vein of Galen aneurysmal malformation (VGAM) causes a compensatory increase in blood volume and cardiac output and lead to High output cardiac failure [2]. in our case the presentation was different due to right Superior Vena Cava (RSVC)-LA, that leads to hemodynamic modification in the form of central cyanosis and increased left ventricular end diastolic pressure, and pulmonary venous congestion

Summary

Conclusions

The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.

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