Late Presentation of Persistent Left Superior Caval Vein in
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Introduction
A persistent left superior caval vein with a reported incidence of 0.3% to 0.5% in the general population is considered the most common congenital venous anomaly of the thoracic venous return. It has been reported in up to 12% of individuals with congenital heart abnormalities, such as septal defects, aortic coarctation, transposition of the great vessels, tetralogy of Fallot, and anomalous connections of the pulmonary veins.

The persistent left superior caval vein usually drains into the right atrium via the coronary sinus. In the rare case of coronary sinus ostial atresia, the left superior caval vein drains the coronary venous blood flow from the coronary sinus to the systemic venous circulation. These anomalies cause no shunts. However, in 10–20% of cases, it drains to the left atrium either via unroofed coronary sinus, or in a straight line fashion into the roof of the left atrium, or the left superior pulmonary vein. It is in this kind of abnormal connection where there is shunting that it may need to be occluded or ligated. Presentation of left superior caval vein in patients following single ventricle palliation has been reported several years after surgery. We report a case where this anomaly was detected 18 years after surgery and was treated successfully by cardiac catheterization.

Case Report
A 35-year-old man with the diagnosis of mitral atresia, a double outlet right ventricle, pulmonary atresia with interrupted inferior caval vein, and azygous continuation to the right superior caval vein underwent a Blalock-Taussig shunt in early childhood and a Kawashima operation at the age of 17 at another institution. He presented to our clinic with progressive cyanosis and fatigue 18 years after his last surgical procedure. His oxygen saturation was 65%.....

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4241290/
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