Surgical and Endovascular Treatment of Symptomatic Aberrant Right Subclavian Artery (Arteria Lusoria)

Reinhard Kopp; Ingrid Wizgall; Eckart Kreuzer; Georgios Meimarakis; Rolf Weidenhagen; Andreas Kühnl; Claudius Conrad; Karl Walter Jauch; Lutz Lauterjung

Disclosures

Vascular. 2007;15(2):84-91. 

In This Article

Discussion

The right aberrant subclavian artery is reported to be the most common intrathoracic abnormality of the aortic arch.[1,2,19] The first successful repair of an aberrant right subclavian artery was reported by Gross.[20] However, the best surgical approach to treat patients with pathologies of the lusorian artery is still a point of discussion. In patients with dysphagia, usually, proximal and distal occlusion of the lusorian artery with mobilization and collapse of the retroesophageal course or complete proximal resection of the lusorian artery is recommended to reduce esophageal compression.[5,8,18] As an alternative, a right supraclavicular approach was reported by Carrizo and Marjani in a symptomatic patient with dysphagia and a nonaneurysmal lusorian artery.[21] For patients with an aneurysmal lusorian artery aneurysm, exclusion is performed by either right or left thoracotomy, sternotomy, or transaortic repair with extracorporal circulation according to the underlying pathology and the site of the aortic arch.[5,8,22,23]

Kieffer and colleagues reported the largest single-center series with 33 patients treated for a symptomatic or aneurysmal lusorian artery.[8] The authors also reported a classification of anomalies of the lusorian artery by separation of their patients into four groups: those with dysphagia lusoria without aneurysm, patients with symptomatic occlusive disease of the lusorian artery, patients with aneurysmal lusorian artery, and those with a thoracic aortic lesion, usually an aneurysm, involving the origin of the lusorian artery. Based on their data, patients were treated according to the underlying pathology, with proximal occlusion of the lusorian artery either by a cervical approach, median sternotomy, or left- or right-sided thoracotomy, according to the side of the aortic arch. The perioperative mortality rate in the group of patients with aneurysmal disease of the lusorian artery or an aneurysm at the aortic origin of the lusorian artery was 23.5%.

Endovascular treatment of peripheral arteries or aneurysms of the thoracic or abdominal aorta has become an important additional treatment option.[9–11,24] The endovascular or combined surgical and endovascular treatment of patients with symptomatic pathologies of the lusorian artery has so far been reported only in six case reports with short follow-up periods, between 6 and 12 months.[12–17] Based on our experience and the reports in the literature, the combined endovascular occlusion of the aortic origin of the lusorian artery with subclavian artery transposition and distal prevertebral occlusion seems to be a safe and effective alternative for elective treatment, with no perioperative deaths so far.

Long-term results, however, are still missing. Nevertheless, follow-up of our two patients reported herein indicated at least promising midterm results, with symptom-free intervals of 82 and 92 months.

Transbrachial stenting in patients with symptomatic lusorian artery stenosis was reported as an additional successful treatment option.[17] However, endoluminal implantation of either an uncovered or a covered stent within the lusorian artery for the treatment of obstructive vascular disease or small aneurysms with close contact to the esophagus or trachea will substantially increase the already imminent risk of the development of arterioesophageal or arteriotracheal fistulae,[25] as shown in our last patient. This approach should therefore be performed only in patients with short ostial stenosis and is not recommended for patients with large lusorian artery aneurysms, a long and broad aneurysmal basis, or a lusorian artery arising from a Kommerell's diverticulum.

Occlusion of the proximal right subclavian artery without subclavian artery reconstruction might cause right arm ischemia or symptoms described as subclavian steal syndrome.[26,27] Although the experience with thoracic stent graft implantation in the proximal descending aorta with occlusion of the left subclavian artery suggests sufficient blood supply to the left arm without transposition of the left subclavian artery,[28] the combined surgical and endovascular approach described for the treatment of right aberrant subclavian artery aneurysms necessitates right subclavian artery transposition since overstenting of the left subclavian artery is frequently required for secure proximal stent graft fixation and might thereby reduce the cerebrobasilar blood flow. In addition, the variability of the differential origins of the supra-aortic carotid and vertebral arteries and the presence of a coronary bypass using the internal mammary artery must be considered.

To our knowledge, operative treatment of an aneurysmal lusorian artery should be considered in every aneurysm larger than 2 cm. In an analysis of 37 lusorian artery aneurysms reported in the literature, we found a pre- and intraoperative rupture rate of 22.6% associated with a mortality rate of 100%, independent of the aneurysm diameter (range 2–10 cm).[18] Perioperative mortality in patients treated by open surgical procedures was 26.9%. All patients with hematemesis and clinical symptoms or evidence of an arterioesophageal fistula died perioperatively.

Three of our four patients with a lusorian artery aneurysm had long-lasting arterial hypertension and have had additional operative treatment for their abdominal aortic aneurysm. According to the reports in the literature, the frequency of an association between a lusorian artery aneurysm and an abdominal aortic aneurysm is between 10 and 20%.[4,8,18] Therefore, patients with a known aberrant lusorian artery should have continuous surveillance and treatment of arterial hypertension and screening for abdominal aortic aneurysms.

In summary, the anomalous course of an aberrant right subclavian artery can cause relevant symptoms owing to compression of the esophagus or the trachea in association with either a nonaneurysmal artery or the development of a lusorian artery aneurysm. Operative repair by either open surgery or in combination with endovascular procedures is recommended to treat symptoms and reduce the risk of aneurysm rupture.

Symptomatic patients with nonaneurysmal lusorian arteries and dysphagia or dyspnea can be treated by either a supraclavicular or transthoracic repair or an extrathoracic cervical-endovascular approach with transbrachial proximal stent graft occlusion.

Intermittent right arm ischemia caused by a stenotic lusorian artery can be treated by simple transbrachial angioplasty or right subclavian artery transposition. Transbrachial endoluminal stent procedures should be avoided and restricted only to short proximal ostial stenoses.

For patients with aneurysms of the lusorian artery or an aneurysm of the proximal descending aorta, endovascular occlusion of the origin of the aberrant subclavian artery by thoracic aortic stent graft implantation in combination with distal occlusion of the lusorian artery and subclavian artery transposition seems to represent an additional minimally invasive approach with promising midterm results. However, experience with endovascular therapy of patients with symptomatic aberrant right subclavian arteries is still limited; therefore, long-term results within a larger group of patients will have to be considered.

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