A 17-year-old male with no previous medical illness was admitted to the hospital with abdominal pain. The patient had a long time history of generalized intermittent abdominal pain that increased postprandial without nausea or vomiting. The patient also had a history of fatigue on exertion. Upon checking vital signs, the patient was found to be hypertensive. There was a discrepancy between the upper (155-160/70-75 mmHg) and lower limbs (100-105/65-70 mmHg) readings. Cardiovascular examination showed a systolic ejection murmur best appreciated at the tip of the left scapula, and bracheo-femoral delay. The abdominal examination was unremarkable. His complete blood count, renal profile, liver profile, troponin, and brain natriuretic peptide were normal. Stool analysis and culture were normal. Electrocardiography revealed sinus rhythm and left ventricular (LV) hypertrophy (Figure ). The chest X-ray revealed the classic rib notching sign (Figure ). An echocardiogram revealed a BAV with mild to moderate aortic regurgitation and dilatation of ascending aorta (Figure ). A post-ductal CoA was observed with a systolic gradient of 80 mmHg (Figure ). His LV function was normal with mild eccentric hypertrophy. Cardiac CT was done and confirmed severe post-ductal CoA with extensive collaterals (Figure ). Another incidental finding was a left-sided IVC, which courses anterior to the abdominal aorta between the aorta itself and superior mesenteric artery crossing the midline to the right before joining the right renal vein and the intra-hepatic IVC segment (Figure ). The patient was discussed in a heart team meeting, and it was decided to proceed with transcatheter intervention with stenting.\nThe procedure was done under general anesthesia through the right femoral access. The pre-stenting peak gradient was 60 mmHg, which improved to 4 mmHg using 14 x 39 mm BeGraft aortic stent (Bentley InnoMed, Hechingen, Germany), which is a balloon-expandable covered stent. This was followed by post-stenting dilation with short non-compliant Z-Med balloon (B. Braun Interventional Systems, Bethlehem, PA) (Figure ). No temporary pacing was used for stenting. No periprocedural complications were encountered. The patient stayed in the hospital for two days, then was eventually discharged home on losartan 100 mg daily. Six weeks later, the patient was called for follow up. His symptoms resolved utterly, and blood pressure was well controlled.
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.