Abstract

Description of Case: A 38-year-old man with known type B interrupted aortic arch (IAA), bicuspid aortic valve, and a complex surgical history (including IAA repair with an ascending-descending aortic conduit, later conduit graft revision, and left pulmonary artery [LPA] stent) presented after many years of loss to follow-up and was found to have hypertensive urgency. Echocardiogram showed turbulent flow in the proximal descending aorta concerning for arch obstruction. Computed tomography angiography (CTA) (Fig. 1 A–C) showed: narrowed proximal graft anastomosis, dilation of the aortic root and ascending aorta, a large pseudoaneurysm arising from the distal graft anastomosis, and LPA stenosis due to compression from the descending aorta. Following improved hypertension, the patient underwent placement of a left carotid-subclavian bypass graft followed by thoracic endovascular aortic repair (TEVAR) with an endograft positioned across the distal aortic jump graft suture line. CTA one month postoperatively (Fig. 1 D–F) confirmed exclusion of the pseudoaneurysm. Discussion: Type B IAA is considered the most common subtype of IAA among cases diagnosed in infancy, though recent data regarding IAA epidemiology are scarce. IAA is associated with other congenital abnormalities, such as ventricular septal defect (VSD), LV outflow tract abnormalities, and 22q11 deletion syndrome. Additional surgical aortic arch procedures in these patients are not uncommon, perhaps especially among patients who required reintervention for LV outflow tract obstruction. While most studies examining long-term outcomes after IAA repair have not commented specifically on late risk of aneurysm or pseudoaneurysm, such findings have been described in the literature. Additionally, use of polytetrafluoroethylene material in arch repair and arch repair by a method other than direct anastomosis—such as were seen in our patient—have been observed to be risk factors for late reintervention.

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