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 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.