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.
Our patient was an 18-year-old girl with recent onset right leg pain and swelling with extension to her inguinal area. She also had dyspnea with respiratory distress. At the time of presentation, she was ill and febrile. She also had pitting edema of right leg and significant size difference between circumferences of her two legs. On the day of admission, she suddenly presented with dyspnea and severe thoracic pain on the right side. All other physical examination was normal.\nShe had no predisposing factor for venous thrombosis. Besides, she had no known a history of DVT, clotting disorders, recurrent abortion, peripheral vascular disease, non-healing ulcerations on the extremities, cerebrovascular accidents or taking oral contraceptive pills.\nHer laboratory data showed only mild normochromic, normocytic anemia, elevated erythrocyte sedimentation rate (ESR) to 75 (normal range less than 25) and CRP = 16 (normal less than 8). Other laboratory test including renal, electrolytes, liver, and urine analysis and culture were normal.\nColor duplex ultrasound study demonstrated deep venous thrombi in the right popliteal, superficial and common femoral veins, with external iliac vein.\nIn addition, hypercoagulable workup was revealed no abnormal finding and also negative for antiphospholipid syndrome and lupus anticoagulant antibodies.\nA multi-detector computed tomography (CT) pulmonary angiography of the chest defined pulmonary emboli in the territory of right main and segmental branch pulmonary artery and suggesting extensive infarction of the right middle pulmonary lobe. She was subsequently started on a low molecular weight heparin (Clexane®: 60 mg, every 12 h SC). Subsequently, CT of the abdomen and CT angiography with IV contrast injection carried out after the procedure confirmed duplication of the IVC []. Both two IVC originate in the pelvis, run parallel to each other, bordering the aorta and left-sided IVC drained into the left renal vein. There was no other pathologic finding in thoracic and abdominopelvic CT studying.\nAfter fully anticoagulated with low molecular weight heparin for 5 days and starting warfarin international normalized ratio (INR) range was between 2 and 3, she was discharged from hospital and was not symptomatic in her follow-up until next 3 months. According to the previous studies, for this patient we planned to continue anticoagulant therapy for 6 months.[]