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

Gulati, Rashmi; Smith, Trevor; Siow, Maria; Batten, Lynn

doi:10.1111/echo.14636

Cited by 1 publication

(1 citation statement)

References 35 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This shunting of deoxygenated blood to the systemic arterial circulation then leads to unexplained persistent cyanosis or hypoxemia of varying severity. To date, there are only eleven children [1,2,3,5,6,9,10] and sixteen adults reported in the literature [3,4,5,7,8]. Aside from aforementioned cyanosis, these patients are generally asymptomatic.…”

Section: Introductionmentioning

confidence: 99%

Delayed Diagnosis of Rare Cyanotic Cardiac Lesion in A Hypoxemic but otherwise Asymptomatic Infant

Sahadan

Ooi

2020

MJPCH

View full text Add to dashboard Cite

The causes of neonatal hypoxemia are usually either pulmonary or cardiac pathologies. We report a case of a 2-month-old boy with oxygen dependency since birth. He would desaturate down to 88-90% whenever supplemental oxygen was weaned off. Initial screening echocardiograms described only a small atrial septal defect, thus lung disease was thought to be the aetiology. Eventually, a detailed echocardiogram and CT angiography revealed anomalous right superior vena cava (RSVC) draining into the left atrium (LA). Echocardiography with bubble study injected via the upper extremities showed brisk filling of “bubbles” in the left heart, confirming fixed right-to-left shunting. Anomalous RSVC drainage into the LA is an extremely rare form of anomalous systemic venous drainage whereby unexplained hypoxemia is present and is sometimes the only clinical manifestation. Surgical correction of this anomaly is indicated to prevent complications of cyanosis and risk of systemic embolization. The surgery generally carries low risk and is associated with good long-term prognosis.

show abstract