C oarctation of the aorta accounts for 3% to 5% of congenital cardiac malformations.1-3 Late aneurysmal formation in the proximal or distal aortic arch is a well-recognized sequela of untreated coarctation, and it is associated with an increased risk of aortic rupture and death. 4 The prevalence of these aneurysms was 20% in the era before surgical treatment was feasible, and 5% thereafter.1,2 It has been reported that 32% of aneurysms are proximal to the coarctation, 51% are distal, and 17% involve the left subclavian artery (LSA) rather than the aorta.1,2 After surgical correction of coarctation, aneurysms may also form at the site of repair, regardless of the surgical technique used.
2We describe the case of a Jehovah's Witness with an untreated coarctation who had an acute dissection of a prestenotic thoracic aortic aneurysm. To avoid open surgery and the need for blood products, we performed an emergency endovascular aortic repair. Only a few patients have undergone this treatment, and, to our knowledge, our patient was the first to have it in an emergency.
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Case ReportIn April 2013, a 44-year-old man with untreated coarctation of the aorta experienced new-onset chest pain and sought treatment at a local hospital. He was transferred to our institution when he was diagnosed with acute dissection of a thoracic aortic aneurysm.The coarctation and a large prestenotic aortic aneurysm, proximal to the LSA, had been diagnosed 20 years earlier. The patient also had a history of systemic hypertension, which had been managed medically. His blood pressure was 150/100 mmHg at the current presentation.Contrast-enhanced computed tomograms showed a tortuous descending thoracic aorta with a voluminous dissected aneurysm, distal to the origin of the subclavian artery ( Figs. 1 and 2). Angiograms showed that the aneurysm was prestenotic (diameter, 78 mm; gradient, 45 mmHg) and proximal to the untreated coarctation (Fig. 3).The patient would have been a candidate for open surgery, but his religious beliefs precluded blood transfusion if bleeding occurred, placing him at an extremely high risk of death. Although endovascular repair has risks, this option was considered to be safer because it would minimize the risk of bleeding and reduce the risk of injury to the laryngeal and phrenic nerves.