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 48-year-old male patient underwent surgical correction of interrupted CoA at the age of 22 years using EABG from the LSCA to the descending aorta. He presented with shortness of breath. The patient was found to be hypertensive with a BP of 170/90 mmHg on both arms. The BP in the lower limbs was 125/85 mmHg with obvious brachiofemoral delay. The patient was taking amlodipine 10 mg and valsartan 160 mg, both once a day. The ECG revealed LV hypertrophy. His TTE revealed normal LV systolic function, with LV hypertrophy and impaired relaxation. A CT angiography revealed stenosis at the proximal anastomosis site of the EABG (Figure ).\nThe plan was to proceed with cardiac catheterization and stenting the proximal stenosis after measuring the gradient but the patient refused any kind of intervention. The patient was sent home after adding a third antihypertensive medication, which wass bisoprolol 5 mg once a day. After six months, the patient's symptoms improved and his BP was 145/80 mmHg. His repeated TTE revealed no major changes.