Isolated drainage of right superior vena cava into left atrium: A rare etiology of childhood central cyanosis

Abstract

Isolated right superior vena cava drainage into the left atrium (RSVC-LA) is an extremely rare anomaly in the absence of other cardiac or extracardiac abnormalities. The clinical and echocardiographic diagnosis is considered challenging. We report a female infant with cyanosis on crying who was diagnosed as isolated RSVC-LA using conventional and contrast echocardiography, but further confirmation was possible via multidetector CT. The surgical correction was performed with smooth postoperative recovery. In conclusion, isolated RSVC-LA must be in the differential diagnosis of cyanosis in the absence of intracardiac anomalies. Surgical correction is necessary management to guard against brain abscesses or paradoxical embolization.

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

“…It is a rare cyanotic CHD and extremely rare when presented as isolated CHD. 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.…”

“…Treatment of RSVC-LA is a surgical repair that must be performed as early as possible to prevent paradoxical embolization and the risk of brain abscesses [ 3 ]. The significant co-morbidities in our patient like severe and poorly respond FTT, recurrent pulmonary infection, and presence of two autosomal dominant diseases, resulted in postponing the surgical correction.…”

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

“…The drainage of a RSVC to LA can be detected in about 0.5% of congenital heart anomalies 2 . The hemodynamic effect of this anomaly is approximately one‐third of systemic venous return causing left‐sided volume overload with resulting hypoxia 3 and the possibility of brain abscesses and strokes 4 . The first case of a RSVC draining into LA was reported in 1956 in a 10‐year‐old female who presented with cyanosis and exercise intolerance with normal CXR and left ventricular hypertrophy on electrocardiogram.…”

“…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.…”