Endovascular Treatment of Distal Aortic Arch Aneurysm Associated with Coarctation of Aorta in a Jehovah's Witness

Abstract

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. 3,5 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.

Saccular “Cauliflower” aneurysm of coarctation of aorta: a hybrid management

“…5 Surgical correction of such aneurysms poses significant challenges of bleeding and risks of rupture. 3 This may be further complicated by the aneurysm being located adjacent to the left subclavian artery. 5 Our case highlights the role of hybrid procedures for managing this situation and may be considered an alternative to open surgery.…”

“…6 The distribution of the aneurysms are as follows: 32% proximal to the coarctation, 51% distal, and 17% involve the left subclavian artery rather than the aorta. 3,6 Inflammation of the aortic wall, congenital weakness of the aortic arterial wall, or asymmetric and enhanced shear stress may play a significant role in the development of aneurysm. 1 A 16-month-old female was described with a saccular aortic aneurysm distal to her aortic coarctation without any history of intervention or vascular inflammatory disease.…”

“…7 Endovascular approach has been previously described in a 44-year-old man with Jehovah's Witness who had unrepaired coarctation of the aorta and presented with a pre-stenotic dissecting thoracic aortic aneurysm. 3 An endovascular aortic repair in which two stent grafts were placed in the descending aorta was successfully performed. Another case reported a 46-year-old man with coarctation of aorta concurrent with aortic arch aneurysm invading the left subclavian artery in whom a novel fenestration device was utilised to create fenestration of left common carotid artery.…”

“…2 Presence of aneurysm in patients with coarctation of the aorta has management challenges and poses high risk of aortic rupture. 3,4 We present a case of coarctation of the aorta diagnosed in a teenage girl with the combination of an aortic arch coarctation, a "cauliflower-like" saccular aneurysm, and stenosis at the origin of the left subclavian artery, which was successfully treated with a hybrid approach.…”

<p>Coarctation of Aorta and Post-Stenotic Dissecting Aortic Aneurysm Successfully Treated with Endovascular Stent</p>

“…In literature, there are few reports of TEVAR for coarctation associated with dissected aneurysm. 6 , 7 …”