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%.\nCardiac catheterization revealed left pulmonary artery stenosis and a decompressing vein from the right superior caval vein to the left superior caval vein which directly drains to the left atrium. Left pulmonary artery stenting was done via right internal jugular approach (Figures , , and ).\nHe underwent a second catheterization for occlusion of the left superior caval vein via left internal jugular approach. The anomalous vein measured 22 and 20 mm in AP and lateral projections, respectively. Balloon test occlusion resulted in immediate increase in oxygen saturation from 65% to 82% without drop of blood pressure. The mean pressure in the azygous vein remained at 9 mmHg; pressure in the left internal jugular vein increased to 11 mmHg and dropped within a few minutes to 10 mmHg. Angiography showed good venous collateral drainage.\nThe largest available vascular plug, 24 mm in diameter, failed to completely occlude the vessel, which was successfully occluded using a 24 mm ASD occluder (Cera Lifetech, China) via a 12 Fr sheath. Upon completion of the procedure the patient had oxygen saturation of 80%, left internal jugular pressure of 10 mmHg, and right superior caval vein and pulmonary artery pressure of 9 mmHg.\nAfter occlusion, the patient had significant improvement of his exercise intolerance and fatigue. Follow-up hemodynamic study 12 months later showed no increase in venous pressure, no development of venous collaterals, and no decrease in his oxygen saturation.
A 30-year-old asymptomatic female with a history of surgically repaired aortic coarctation presented for routine follow-up. Chest computed tomography angiography (CTA) demonstrated a left-sided IVC with hemiazygos continuation to the left-sided SVC in the absence of right-sided SVC (Figure ), a very rare systemic venous anomaly. The left-sided superior vena cava was draining to the enlarged coronary sinus, and the hepatic veins were directly connected to the right atrium (Figure ).\nThe case has been followed up for the coarctation without any treatment regarding to venous anomaly.