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

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.

Isolated right superior vena cava drained to left atrium in a child with Waardenburg syndrome and neurofibromatosis type I

“…Worldwide incidence is less than 0.5% of all CHD [ 3 ]. Similar cases were reported in KSA [ 4 , 5 ]; however, the actual national incidence is not known.…”

Isolated right superior vena cava drainage into the left atrium: An uncommon cause of persistent hypoxia in a neonate

“…There are several cases of this defect in association with other congenital heart defects and heterotaxy, but through our review only 42 cases of this as an isolated anomaly have been reported in the English literature to date 1,3‐43 . Table 1 summarizes the reported cases as case number, year of publication, first author, age and gender of patient, presentation, method of diagnosis, and the outcome.…”

Vein of Galen malformations in the newborn: case series

“…VoGMs are exceptionally rare with an incidence of only 1 in 3 million. [36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51] Therefore, we elected to supplement our case series with additional cases from the literature to further characterize VoGMs subtypes I-IV in greater detail. This constitutes the second major limitation, namely selection bias, because authors generally tend to report their better cases only.…”