A 28-year-old man, with a history of CoA repair using Dacron patch aortoplasty at the age of 10 years, presented with shortness of breath and sudden, severe central chest pain that radiated across his chest and back. The patient had been lost to clinical follow-up over the last 15 years. He was conscious, hypotensive (90/60 mmHg on both arms), and in distress with tachycardia (120 BPM) and relatively weak pedal pulses with cold distal extremities. A chest X-ray (CXR) demonstrated the widening of the upper mediastinum (Figure ).\nHe underwent emergency CT angiography, which demonstrated extensive mediastinal hematoma and contrast leaking from a 4x12 cm complex pseudoaneurysm of the proximal thoracic descending aorta (Figure ).\nThe intimal disruption started about 2 cm distal to the origin of the left subclavian artery (LSCA), which was probably the site of the patch repair. The aortic diameter just prior to the disruption was 2.5 cm, and the normal distal thoracic aortic diameter was 2 cm. Of note, there was compression to the distal aorta by the pseudoaneurysm, leading to significant narrowing, which explained the weak distal pulses and cold extremities. After the heart-team discussion, the decision was made to proceed with transcatheter endograft implantation. The transcatheter approach was deemed more feasible and safer than a surgical one, especially in the presence of a good landing zone for endograft placement (away from the LSCA) (Figure ).\nThe patient was taken to cardiac catheterization laboratory and, under general anesthesia, we implanted a tapered (28 mm - 26 mm) x 150 mm Valiant Thoracic Stent Graft with the Captivia Delivery System (Medtronic Vascular, Santa Rosa, CA). The endograft was implanted successfully with complete exclusion of the pseudoaneurysm and excellent flow distally (Figure ).\nThe access site (right femoral artery) was secured successfully with two Perclose ProGlide™ devices (Abbott Laboratories, Abbott Park, IL). The patient did very well and was discharged home after three days. A follow-up CT angiography revealed a widely patent stent graft with no endoleaks.
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.