Subclavian Artery Aneurysms in Marfan Syndrome: A Clinical Dilemma
Presented During:
Thursday, April 25, 2024: 5:38PM - 7:00PM
Sheraton Times Square
Posted Room Name:
Central Park
Abstract No:
P0312
Submission Type:
Abstract Submission
Authors:
Adam Carroll (1), Lance Dzubinski (1), Rafael Malgor (1), Donald Jacobs (1), Muhammad Aftab (1), T. Brett Reece (1)
Institutions:
(1) University of Colorado Anschutz, Denver, CO
Submitting Author:
Adam Carroll
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University of Colorado Anschutz
University of Colorado Anschutz
Co-Author(s):
LANCE DZUBINSKI
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University of Colorado Anschutz
University of Colorado Anschutz
Rafael Malgor
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University of Colorado Anschutz
University of Colorado Anschutz
Donald Jacobs
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University of Colorado Anschutz
University of Colorado Anschutz
*Muhammad Aftab
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University of Colorado Anschutz
University of Colorado Anschutz
*T. Brett Reece
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University of Colorado Anschutz
University of Colorado Anschutz
Presenting Author:
LANCE DZUBINSKI
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University of Colorado Anschutz
University of Colorado Anschutz
Abstract:
Objective:
Cardiovascular manifestations of Marfan syndrome typically present as aortopathy or valvular disease. Rarely, they can present with peripheral aneurysms. Subclavian artery aneurysms (SCAA) are particularly rare, representing <1% of all peripheral aneurysms with connective tissue patients accounting for <10% of SAA. We discuss a familial case of Marfan syndrome in two patients with a history of extensive aortopathy that in turn developed subclavian artery aneurysms.
Methods:
We discuss two patients with Marfan syndrome and SCAA, a 37-year-old male (Patient 1) and his 57-year-old mother (Patient 2). We review their relevant prior history, presentation, and management.
Results:
Patient 1 presented to our institution at age 32, with a history of a previous aortic root and mitral valve replacement and an enlarging type B dissection involving the distal arch and left subclavian artery (LSCA) extending to his left iliacs. A staged repair was planned, including a left carotid-SCA bypass, ligation of the LSCA, and elephant trunk arch replacement. This was followed by an extent 2 thoracoabdominal aortic repair with aorto-bi-iliac bypasses.
Two years following, he developed enlargement of his bilateral axillary artery (AA) aneurysms (R 4.5[3.3]cm, L 2.3[0.8]cm), and enlargement of the LSCA (2.3[1.5]cm). He first underwent a right SCA to brachial artery bypass. His LSCAA appeared to include several branches, including the vertebral artery, placing the patient at risk for operative stroke. Coil embolization of the vertebral was performed. No other aneurysm branch vessels were able to be cannulated, and a vascular plug was placed at the neck. Angiography demonstrated sac thrombosis. However, post-operative imaging demonstrated ongoing flow to LSCAA, as well as a new large left AA/brachial aneurysm. He also developed left arm pain, with duplex showing minimal left arm flow. Open ligation of the LSCAA, with excision and bypass of the AA aneurysm was performed. The LSCAA was isolated, however, when clamped proximally significant bleeding occurred from the top of the LSCAA. The LSCAA was divided off the bypass and suture closed. The residual LSCA was unsuitable for bypass and was ligated. The residual carotid-SCA bypass was sutured to the AA-brachial graft, with improvement in left arm flow. He was discharged on post-operative day 2 without issue. Despite the extensive interventions above, the LSCAA stump remains with filling from the internal mammary and thyrocervical trunk. Pending approval of new embolic plugs, the patient will undergo further intervention.
Patient 2 presented to our institution at age 57, with a history of previous aortic valve, and ascending aortic, hemiarch, and abdominal aorta repair, with an expanding LSCA and AA aneurysm measuring 4.9 x 7.8cm, causing worsening nerve compression. To exclude collateral branches, she underwent LSCA and AA stenting, in addition to an AA to brachial bypass. The procedure was uncomplicated, however, a year following she presented with acute limb ischemia related to complete occlusion of the stents. She subsequently underwent failed endovascular thrombectomy and thrombolytic therapy, ultimately requiring open thrombectomy and grafting with resolution.
Conclusion:
Marfan patients with SCAA present a challenging clinical dilemma. Given their vasculopathy, they are at high risk of rapid expansion of pathology and require close surveillance and aggressive intervention.
Cardiovascular manifestations of Marfan syndrome typically present as aortopathy or valvular disease. Rarely, they can present with peripheral aneurysms. Subclavian artery aneurysms (SCAA) are particularly rare, representing <1% of all peripheral aneurysms with connective tissue patients accounting for <10% of SAA. We discuss a familial case of Marfan syndrome in two patients with a history of extensive aortopathy that in turn developed subclavian artery aneurysms.
Methods:
We discuss two patients with Marfan syndrome and SCAA, a 37-year-old male (Patient 1) and his 57-year-old mother (Patient 2). We review their relevant prior history, presentation, and management.
Results:
Patient 1 presented to our institution at age 32, with a history of a previous aortic root and mitral valve replacement and an enlarging type B dissection involving the distal arch and left subclavian artery (LSCA) extending to his left iliacs. A staged repair was planned, including a left carotid-SCA bypass, ligation of the LSCA, and elephant trunk arch replacement. This was followed by an extent 2 thoracoabdominal aortic repair with aorto-bi-iliac bypasses.
Two years following, he developed enlargement of his bilateral axillary artery (AA) aneurysms (R 4.5[3.3]cm, L 2.3[0.8]cm), and enlargement of the LSCA (2.3[1.5]cm). He first underwent a right SCA to brachial artery bypass. His LSCAA appeared to include several branches, including the vertebral artery, placing the patient at risk for operative stroke. Coil embolization of the vertebral was performed. No other aneurysm branch vessels were able to be cannulated, and a vascular plug was placed at the neck. Angiography demonstrated sac thrombosis. However, post-operative imaging demonstrated ongoing flow to LSCAA, as well as a new large left AA/brachial aneurysm. He also developed left arm pain, with duplex showing minimal left arm flow. Open ligation of the LSCAA, with excision and bypass of the AA aneurysm was performed. The LSCAA was isolated, however, when clamped proximally significant bleeding occurred from the top of the LSCAA. The LSCAA was divided off the bypass and suture closed. The residual LSCA was unsuitable for bypass and was ligated. The residual carotid-SCA bypass was sutured to the AA-brachial graft, with improvement in left arm flow. He was discharged on post-operative day 2 without issue. Despite the extensive interventions above, the LSCAA stump remains with filling from the internal mammary and thyrocervical trunk. Pending approval of new embolic plugs, the patient will undergo further intervention.
Patient 2 presented to our institution at age 57, with a history of previous aortic valve, and ascending aortic, hemiarch, and abdominal aorta repair, with an expanding LSCA and AA aneurysm measuring 4.9 x 7.8cm, causing worsening nerve compression. To exclude collateral branches, she underwent LSCA and AA stenting, in addition to an AA to brachial bypass. The procedure was uncomplicated, however, a year following she presented with acute limb ischemia related to complete occlusion of the stents. She subsequently underwent failed endovascular thrombectomy and thrombolytic therapy, ultimately requiring open thrombectomy and grafting with resolution.
Conclusion:
Marfan patients with SCAA present a challenging clinical dilemma. Given their vasculopathy, they are at high risk of rapid expansion of pathology and require close surveillance and aggressive intervention.
Aortic Symposium:
Genetics of Aortic Disease
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