Abstract

Clinical Summary A 31-year-old woman was admitted to a peripheral hospital with an episode of massive hematemesis. She had undergone a patchtechnique coarctation repair at age 3 years and required balloon dilatation for recoarctation at age 14 years. Annual cardiologic follow-up detected, at age 26 years, a pseudoaneurysm at the coarctation site with dimensions of 2.4 1.9 cm. The pseudoaneurysm size remained stable, but 8 months before the hematemesis, TEVAR was recommended to avert any risk of rupture and a Jomed 48 20-mm covered stent (Abbott Vascular Ltd, Kent, United Kingdom) was deployed at the coarctation site pseudoaneurysm. Post-TEVAR imaging suggested satisfactory exclusion. After the hematemesis, she underwent esophagogastroduodenoscopy, which showed a 1.5-cm bluish polypoid lesion 26 cm from the incisura. Biopsy revealed blood clot only. She was discharged but readmitted 4 weeks later with a further massive hematemesis. A repeat esophagogastroduodenoscopy revealed that the lesion had enlarged 3-fold. This finding, together with the history of coarctation surgery, prompted a computed tomographic (CT) scan (Figure 1), which demonstrated a perigraft hematoma suggestive of AEF. After transfer to our center, aortography confirmed peri–stent-graft extravasation, further supporting a diagnosis of AEF. Another covered stent (38 12 mm, Jostent; Abbott Vascular Ltd) was deployed and the hematemesis abated. However, a postprocedure angiogram demonstrated persistent filling of the pseudoaneurysm. The incomplete pseudoaneurysm exclusion and the perceived risk of further complications prompted surgical referral. Echocardiography demonstrated moderate bicuspid aortic valve stenosis. Surgery was performed via a transsternal thoracotomy in the left fourth intercostal space and comprised mechanical aortic valve replacement, retrocardiac ascending–descending aortic conduit insertion, and an interposition graft between the conduit and the left subclavian artery. The aortic arch was staple-ligated between the left common carotid artery and the left subclavian artery. The descending aorta was stapled below the stent site. The aortic pseudoaneurysm was opened and the previously placed stents were removed. Inspection revealed a section of necrotic and partially absent aortic wall with erosion of the longitudinal esophageal muscle but no mucosal breach. It was assumed that stent deployment had perforated or disintegrated the aortic wall leading to an AEF. The aortic remnant was left open, a feeding enterostomy and decompression gastrostomy performed, and the chest closed with drainage. Recovery was uneventful, with a contrast swallow examination showing no abnormalities. At 12 months, the patient remains free of symptoms, and CT scan (Figure 2) demonstrates resolution of perigraft hematoma and satisfactory anatomic reconfiguration.

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