Bilateral subclavian artery aneurysm in a patient with marfan's syndrome

Abstract

Introduction: Subclavian artery aneurysms (SAA) are uncommon and usually caused by thoracic outlet syndrome and
atherosclerosis. Despite the common occurrence of aortic aneurysms in patients with Marfan's syndrome (MFS), SAA are
not a typical finding unless aortic dissection with involvement of the supraaortic vessels has occurred.
Methods: A 23-year-old Marfan patient with a history of several prior aortic procedures due to aortic valve insufficiency
and various dilatations of the aorta but not dissection presented with bilateral SAA during follow-up. CT-angiography
(CTA) revealed de novo aneurysm of the total aortic arch, the proximal descending aorta and the maximal diameters of
both subclavian arteries were 5 cm (Figure A). Echocardiography showed severe aortic regurgitation. The decision for
elective surgical correction as a re-re-do operation was taken. After replacing the aortic valve with a mechanical
prosthesis and cooling down to 26 °C, the aortic arch was opened during hypothermic circulatory arrest. To replace the
aortic arch and proximal descending aorta, a hybrid graft (Thoraflex Hybrid, Vascutek Deutschland GmbH) was used.
Both SAA were excised and the vertebral arteries were prepared for later re-implantation. The subclavian arteries were
reconstructed with end-to-end anastomoses to the branches of the hybrid prosthesis. Both vertebral arteries were reimplanted into the subclavian branches using autologous saphenous vein grafts. After a prolonged recovery due to
postoperative bleeding and subsequent rethoracotomy, the patient was discharged on the 27th postoperative day. Twelve
months follow-up, the patient had well recovered with a satisfying physical state and quality of life. Follow-up CTA
displayed no reoccurence of SAA (Figure B).
Conclusion: Multiple reoperations for aneurysms in Marfan-patients and surgical correction of SAA are feasible. Our
case demonstrates that reestablishment of the regular anatomy of aortic arch and supraaortic vessels is difficult but can
be successfully performed in patients with MFS.

Full text

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N. Firat Sipahi
Department of Cardiac Surgery, University Hospital Munich

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N. Firat Sipahi
Department of Cardiac Surgery, University Hospital Munich