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Hybrid Repair of Bilateral Subclavian Artery Aneurysms in a Patient with Marfan Syndrome
Hybrid Repair of Bilateral Subclavian Artery Aneurysms in a Patient with Marfan Syndrome Jonathan N. Bowman, Sharif H. Ellozy, Konstadinos Plestis, Michael L. Marin, and Peter L. Faries, New York, New York
A 32-year-old man with Marfan syndrome presented with enlarging, asymptomatic bilateral subclavian artery aneurysms. He has an extensive surgical history including aortic arch and descending thoracic aorta replacement. The L aneurysm was treated first with an L carotidevertebral artery vein bypass, aneurysm debranching, and stent-graft repair of aneurysm via the L brachial artery approach. The R aneurysm was treated by placing a stent graft from the proximal R common carotid artery across the R subclavian artery origin and landing in the prosthetic innominate bypass graft via an L common carotid artery conduit. An aneurysm debranching and R carotidesubclavian artery bypass completed the procedure. A proximal type I endoleak was detected in the R aneurysm sac on follow-up computed tomography angiography. This was treated with sternotomy, aorta to L common carotid artery bypass, stent graft removal, and oversewing of the R subclavian artery origin. The patient recovered uneventfully. Subclavian artery aneurysms are rarely diagnosed in patients with Marfan disease. Although durability remains unproven, hybrid repair should be considered in patients with subclavian artery aneurysms, to minimize the morbidity commonly associated with open repair.
True subclavian artery aneurysms are uncommon and account for <1% of all peripheral artery aneurysms.1 Aneurysms of the distal artery are usually associated with thoracic outlet syndrome or trauma.2 Aneurysms of the proximal and middle vessel are usually a result of atherosclerotic degeneration.3 Connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, giant-cell arteritis, and Takayasu arteritis are rarely the etiology. Aneurysms can be asymptomatic or present with compressive symptoms, thrombosis, rupture, or distal embolization. Traditional treatment is open surgical resection of the aneurysm and arterial reconstruction with autogenous or
Presented at the 19th Annual Winter Meeting of the Peripheral Vascular Surgery Society, Steamboat Springs, CO, January 30 February 1, 2009. Department of Surgery, Mount Sinai School of Medicine, New York, NY. Correspondence to: Prof. Jonathan N. Bowman, Department of Surgery, Mount Sinai School of Medicine, 5 E. 98th Street, Box 1273, New York, NY 10029, USA, E mail: [email protected] Ann Vasc Surg 2010; 24: 114.e1 114.e5 DOI: 10.1016/j.avsg.2009.04.005 Ó Annals of Vascular Surgery Inc. Published online: July 23, 2009
synthetic conduit. Patients with connective tissue disorders may have additional morbidity from this procedure due to more rapid arterial deterioration and prior aortic procedures.4 Several reports have demonstrated successful endovascular treatment of subclavian artery aneurysms in Marfan patients.5,6 We present the hybrid repair of bilateral subclavian artery aneurysms in a patient with Marfan syndrome.
CASE REPORT A 32-year-old man with Marfan syndrome presented with enlarging bilateral subclavian artery aneurysms. He had an extensive surgical history. In 1995 he underwent elective repair of a dissection-related abdominal aortic aneurysm with an aorta to bi-iliac synthetic graft. He developed an anastomotic pseudoaneurysm of the R iliac anastomosis in 1996 and underwent stent-graft repair. In April 2003 his aortic valve and aortic arch were replaced electively in a stage I elephant trunk procedure. The etiology of his enlarging aneurysm was chronic dissection. His arch vessels were reconstructed with a trifurcated graft arising from the 114.e1
114.e2 Case reports
ascending portion of his neo-aorta7 (Fig. 1). The second stage of the elephant trunk procedure, repair of his proximal descending thoracic aorta, was completed in May 2003. After recovery from these procedures, he underwent repair of a type IV thoracoabdominal aneurysm related to dissection in October 2003. Diagnostic work-up prior to his arch replacement showed a R subclavian artery dissection but no evidence of a L subclavian aneurysm. On followup imaging after his thoracoabdominal aneurysm repair he was found to have enlargement of the R subclavian dissection and a new L subclavian artery aneurysm. The innominate artery was not diseased. Each aneurysm was <2 cm and asymptomatic. A surveillance computed tomographic angiogram (CTA) in July 2008 showed that the aneurysms had enlarged to 3.1 cm on the R and 2.7 cm on the L. The patient was asymptomatic with palpable radial pulses. Due to the rapid enlargement and patient concern, the decision was made to begin treatment. The L aneurysm was treated first. Preoperative CTA confirmed the dominance of the L vertebral artery and the adequacy of the proximal and distal landing zones in the subclavian artery for stent graft (Fig. 1). The R vertebral artery would be ligated in the second stage. A preoperative head CTA confirmed a patent circle of Willis. A supraclavicular incision was made and an L common carotid artery to vertebral artery bypass with reversed saphenous vein was created. An on-table angiogram confirmed a patent bypass without defect. The proximal vertebral artery, internal mammary artery, and thyrocervical trunk were then ligated. The left brachial artery was then accessed via cutdown. Percutaneous transfemoral access was not possible due to the anatomical constraints created by the prior aortic arch replacement and infrarenal aortic replacement. An angiogram confirmed the patency of the vertebral bypass, with no evidence of remaining feeding branch vessels. Three Viabahn stent grafts (10 mm 8 cm; W. L. Gore and Associates, Flagstaff, AZ) were then placed in the subclavian artery across the aneurysm. Completion angiography showed no evidence of endoleak and a patent vertebral bypass. Fluoroscopy demonstrated no compression of the stent grafts or loss of radial pulse with the arm elevated. The patient had a palpable L radial pulse at completion of the procedure. One month later the patient returned for treatment of the R aneurysm. Prior to the procedure he was advised of all possible complications including endoleak. In conjunction with the cardiac surgeon, he was counseled that if the endovascular treatment
Annals of Vascular Surgery
Fig. 1. Three dimensionally reconstructed CTA of preoperative anatomy. Arrow indicates the origin of the trifurcated graft off the neo aorta. The graft then branches to the L subclavian, the L common carotid, and the innominate artery.
failed, conversion to open repair with sternotomy would be done at this operation. A right supraclavicular incision was made, and the common carotid artery was identified and controlled. A 10 mm Hemashield graft was anastomosed in an end-to-side fashion (Fig. 2). The graft was clamped distally and used as a conduit to deploy a 16 mm 10 mm 7 cm conical Excluder endoprosthesis (contralateral leg of abdominal bifurcated graft, W. L. Gore and Associates). The graft was landed proximally at the origin of the L common carotid artery graft and distally in the common carotid artery (Fig. 2). A small proximal endoleak was seen on postdeployment angiogram and was treated with a second more proximally placed endograft of the same dimensions. The endoleak appeared to be treated, and the decision was made, with the cardiac surgeon, not to convert to open repair. The vertebral artery, thyrocervical trunk, and internal mammary artery were then ligated. The distal end of the Hemashield conduit was anastomosed to the distal subclavian artery in an end-to-end fashion with fenestration of the dissection. The subclavian artery was ligated distal to the aneurysm. The patient had a palpable R radial pulse on completion of the procedure. A postoperative CTA demonstrated complete reconstruction (Fig. 3). A significant proximal type I endoleak was seen on the R (Fig. 4). The patient was presented
Vol. 24, No. 1, January 2010
Fig. 2. Intraoperative angiogram taken during treatment of the R aneurysm. Arrow indicates the location of the R common carotid Hemashield conduit. Inset: Arrow depicts the stent graft being positioned at the distal origin of the L common carotid artery.
with several treatment options and, due to his age and fitness, chose open repair. In conjunction with the cardiothoracic surgeons, the patient underwent redo sternotomy approximately 1 month later. A bypass was created from his aortic arch to the common carotid artery with an 8 mm Dacron graft. The innominate graft was ligated just distal to the take-off of the L common carotid artery. The aneurysm sac was opened, and no back-bleeding was seen. The stent graft was removed, and the subclavian artery origin was oversewn. The patient recovered uneventfully and was discharged home in 6 days. A completion CTA 1 month later showed complete exclusion of both aneurysms without endoleak. The R carotid subclavian artery bypass, L carotid vertebral artery bypass, and aorta R carotid artery bypass were all patent (Fig. 5). The patient will receive the next CTA at 6 months. His follow-up imaging schedule will be based on the results of his CTA at 6 months. The patient is on lifelong coumadin for his mechanical heart valve.
DISCUSSION Subclavian artery aneurysms are uncommon and usually related to atherosclerosis, thoracic outlet syndrome, or trauma.8 Only case reports of
Case reports 114.e3
Fig. 3. Three dimensionally reconstructed CTA after hybrid repair of R and L subclavian artery aneurysms. Arrow shows the patent L carotid to vertebral artery bypass.
subclavian artery aneurysms related to Marfan syndrome exist in the literature4-6,9,10 Patients can present with ischemic complications related to thrombosis or embolization. Rupture is rare, being seen in only two of 31 patients in the series of Pairolero et al.8 Compression-related symptoms can include upper extremity motor and sensory deficits, respiratory distress, hoarseness, Horner syndrome, and dysphagia. Patients commonly complain of upper extremity pain on the affected side.8 A palpable supraclavicular mass and supraclavicular bruit may be found on exam. Our patient was asymptomatic, and his aneurysms were diagnosed on routine follow-up CT scans. Traditional treatment of subclavian artery aneurysms involves sternotomy or thoracotomy with aneurysm resection and restoration of upper extremity perfusion with synthetic or autogenous interposition graft. Long-term patency is excellent; however, morbidity rates can be as high as 46%.8,11 The chance of morbidity may be even higher in Marfan patients due to their propensity for anastomotic breakdown and the possibility of redo aortic surgery. May et al.12 first described exclusion of a subclavian aneurysm with endovascular stent graft. As stent graft and endovascular technology have advanced, the use of stent grafts to treat subclavian aneurysms has become first-line therapy in anatomically appropriate patients due to the lower
114.e4 Case reports
Fig. 4. Axial slice of CTA depicting proximal R innominate portion of stent graft. Arrow indicates an endoleak originating from the proximal seal zone. No evidence of type II endoleak was seen.
morbidity and adequate patency.4,6,13 This technology is even more attractive in patients with connective tissue disorders as it can diminish the need for arterial anastomoses and help avoid reoperative surgery.5 Although endovascular repair has become common, long-term data regarding durability, patency, and maintenance of structure and position in a mobile area are absent. Patients will need more frequent postprocedure imaging studies to ensure adequacy of the repair. Several factors were taken into account when planning this reconstruction. The patient had a prior sternotomy and L thoracotomy, his neo-aortic arch anatomy precluded percutaneous transfemoral treatment, both vertebral artery origins were involved in the aneurysms, the majority of his aorta had been replaced (increasing the risk of spinal cord ischemia with vertebral artery ligation), and no proximal R subclavian landing zone. Also, dissection in the R subclavian artery prevented wire access. Hybrid repair with current endovascular and open technology allowed treatment of the aneurysms with minimal morbidity. This case also highlights some limits of endovascular repair. Despite appropriate sizing and maximal proximal placement of the R-sided stent graft, a type I endoleak was still present. This may have been due to anatomical factors related to the innominate artery anastomosis from the prior aortic arch replacement. Other interventions, such as extension with a bare metal stent covering the L common carotid or coil embolization of the tract, could have
Annals of Vascular Surgery
Fig. 5. Three dimensionally reconstructed CTA of final reconstruction. Arrow indicates a patent aorta to R common carotid artery bypass. Both aneurysms are excluded, with no evidence of endoleak.
been attempted; but a type I endoleak in a young patient requires definitive treatment. Fortunately, this patient was able to tolerate a redo sternotomy without significant morbidity. The combination of current endovascular technology and traditional surgical technique was used to successfully treat this patient with Marfan syndrome and bilateral subclavian artery aneurysms. Endovascular therapy is promising due to the potential for low morbidity. Stent-graft repair in patients with connective tissue disorders may be less durable over time due to arterial dilations at anchor zones. Frequent long-term surveillance is mandatory. REFERENCES 1. Lawrence P, Gazak C, Bhirangi L, et al. The epidemiology of surgically repaired aneurysms in the United States. J Vasc Surg 1999;30:632 640. 2. Scher L, Veith F, Samson R, et al. Vascular complications of thoracic outlet syndrome. J Vasc Surg 1986;3:565 568. 3. Dougherty M, Calligaro K, Savarese R, et al. Atherosclerotic aneurysm of the intrathoracic subclavian artery: a case report and review of the literature. J Vasc Surg 1995;21: 521 529. 4. Kasirajan K, Matteson B, Marek J, et al. Covered stents for true subclavian aneurysm in patients with degenerative connective tissure disorders. J Endovasc Ther 2003;10: 647 652. 5. Gonzalez J, Garcia B, Lebrun J, et al. Combined surgery for the treatment of bilateral subclavian artery aneurysm in Marfan syndrome. J Vasc Surg 2007;45:180 182.
Vol. 24, No. 1, January 2010
6. Tassiopoulos A, Nadalin G, Labropoulos N, et al. Endovascular repair of a symptomatic subclavian artery aneruysm in a patient with Marfan syndrome. J Vasc Endovasc Surg 2006;40:409 413. 7. Etz C, Plestis K, Kari F, et al. Staged repair of thoracic and thoracoabdominal aortic aneurysms using the elephant trunk technique: a consecutive series of 215 first stage and 120 complete repairs. Eur J Cardthorac Surg 2008;34:605 615. 8. Pairolero P, Walks J, Rayne W, et al. Subclavian axillary artery aneurysms. Surgery 1981;90:757 763. 9. Nawa S, Ikeda E, Ichihara S, et al. A true aneurysm of axillarye subcalvian artery with cystic medionecrosis: an unusual min festation of Marfan syndrome. Ann Vasc Surg 2003;17: 562 564.
Case reports 114.e5
10. de Virgilio C, Cherry K, Schaff H. Multiple aneurysms and aortic dissection: an unusual manifestation of Marfan’s syndrome. Ann Vasc Surg 1994;8:383 386. 11. Salo J, Ala Kulju K, Heikkinen L, et al. Diagnosis and treatment of subclavian artery aneurysms. Eur J Vasc Surg 1990;4:271 274. 12. May J, White G, Waugh R, et al. Transluminal placement of a prosthetic graft stent device for treatment of subclavian artery aneurysm. J Vasc Surg 1993;18:1056 1059. 13. Kasirajan K, Matteson B, Marek J, et al. Covered stents for true subclavian aneurysms in patients with degenerative connective tissue disorders. J Endovasc Ther 2003;10: 647 652.
A 32-year-old man with Marfan syndrome presented with enlarging, asymptomatic bilateral subclavian artery aneurysms. He has an extensive surgical history including aortic arch and descending thoracic aorta replacement. The L aneurysm was treated first with an L carotidevertebral artery vein bypass, aneurysm debranching, and stent-graft repair of aneurysm via the L brachial artery approach. The R aneurysm was treated by placing a stent graft from the proximal R common carotid artery across the R subclavian artery origin and landing in the prosthetic innominate bypass graft via an L common carotid artery conduit. An aneurysm debranching and R carotidesubclavian artery bypass completed the procedure. A proximal type I endoleak was detected in the R aneurysm sac on follow-up computed tomography angiography. This was treated with sternotomy, aorta to L common carotid artery bypass, stent graft removal, and oversewing of the R subclavian artery origin. The patient recovered uneventfully. Subclavian artery aneurysms are rarely diagnosed in patients with Marfan disease. Although durability remains unproven, hybrid repair should be considered in patients with subclavian artery aneurysms, to minimize the morbidity commonly associated with open repair.
True subclavian artery aneurysms are uncommon and account for <1% of all peripheral artery aneurysms.1 Aneurysms of the distal artery are usually associated with thoracic outlet syndrome or trauma.2 Aneurysms of the proximal and middle vessel are usually a result of atherosclerotic degeneration.3 Connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, giant-cell arteritis, and Takayasu arteritis are rarely the etiology. Aneurysms can be asymptomatic or present with compressive symptoms, thrombosis, rupture, or distal embolization. Traditional treatment is open surgical resection of the aneurysm and arterial reconstruction with autogenous or
Presented at the 19th Annual Winter Meeting of the Peripheral Vascular Surgery Society, Steamboat Springs, CO, January 30 February 1, 2009. Department of Surgery, Mount Sinai School of Medicine, New York, NY. Correspondence to: Prof. Jonathan N. Bowman, Department of Surgery, Mount Sinai School of Medicine, 5 E. 98th Street, Box 1273, New York, NY 10029, USA, E mail: [email protected] Ann Vasc Surg 2010; 24: 114.e1 114.e5 DOI: 10.1016/j.avsg.2009.04.005 Ó Annals of Vascular Surgery Inc. Published online: July 23, 2009
synthetic conduit. Patients with connective tissue disorders may have additional morbidity from this procedure due to more rapid arterial deterioration and prior aortic procedures.4 Several reports have demonstrated successful endovascular treatment of subclavian artery aneurysms in Marfan patients.5,6 We present the hybrid repair of bilateral subclavian artery aneurysms in a patient with Marfan syndrome.
CASE REPORT A 32-year-old man with Marfan syndrome presented with enlarging bilateral subclavian artery aneurysms. He had an extensive surgical history. In 1995 he underwent elective repair of a dissection-related abdominal aortic aneurysm with an aorta to bi-iliac synthetic graft. He developed an anastomotic pseudoaneurysm of the R iliac anastomosis in 1996 and underwent stent-graft repair. In April 2003 his aortic valve and aortic arch were replaced electively in a stage I elephant trunk procedure. The etiology of his enlarging aneurysm was chronic dissection. His arch vessels were reconstructed with a trifurcated graft arising from the 114.e1
114.e2 Case reports
ascending portion of his neo-aorta7 (Fig. 1). The second stage of the elephant trunk procedure, repair of his proximal descending thoracic aorta, was completed in May 2003. After recovery from these procedures, he underwent repair of a type IV thoracoabdominal aneurysm related to dissection in October 2003. Diagnostic work-up prior to his arch replacement showed a R subclavian artery dissection but no evidence of a L subclavian aneurysm. On followup imaging after his thoracoabdominal aneurysm repair he was found to have enlargement of the R subclavian dissection and a new L subclavian artery aneurysm. The innominate artery was not diseased. Each aneurysm was <2 cm and asymptomatic. A surveillance computed tomographic angiogram (CTA) in July 2008 showed that the aneurysms had enlarged to 3.1 cm on the R and 2.7 cm on the L. The patient was asymptomatic with palpable radial pulses. Due to the rapid enlargement and patient concern, the decision was made to begin treatment. The L aneurysm was treated first. Preoperative CTA confirmed the dominance of the L vertebral artery and the adequacy of the proximal and distal landing zones in the subclavian artery for stent graft (Fig. 1). The R vertebral artery would be ligated in the second stage. A preoperative head CTA confirmed a patent circle of Willis. A supraclavicular incision was made and an L common carotid artery to vertebral artery bypass with reversed saphenous vein was created. An on-table angiogram confirmed a patent bypass without defect. The proximal vertebral artery, internal mammary artery, and thyrocervical trunk were then ligated. The left brachial artery was then accessed via cutdown. Percutaneous transfemoral access was not possible due to the anatomical constraints created by the prior aortic arch replacement and infrarenal aortic replacement. An angiogram confirmed the patency of the vertebral bypass, with no evidence of remaining feeding branch vessels. Three Viabahn stent grafts (10 mm 8 cm; W. L. Gore and Associates, Flagstaff, AZ) were then placed in the subclavian artery across the aneurysm. Completion angiography showed no evidence of endoleak and a patent vertebral bypass. Fluoroscopy demonstrated no compression of the stent grafts or loss of radial pulse with the arm elevated. The patient had a palpable L radial pulse at completion of the procedure. One month later the patient returned for treatment of the R aneurysm. Prior to the procedure he was advised of all possible complications including endoleak. In conjunction with the cardiac surgeon, he was counseled that if the endovascular treatment
Annals of Vascular Surgery
Fig. 1. Three dimensionally reconstructed CTA of preoperative anatomy. Arrow indicates the origin of the trifurcated graft off the neo aorta. The graft then branches to the L subclavian, the L common carotid, and the innominate artery.
failed, conversion to open repair with sternotomy would be done at this operation. A right supraclavicular incision was made, and the common carotid artery was identified and controlled. A 10 mm Hemashield graft was anastomosed in an end-to-side fashion (Fig. 2). The graft was clamped distally and used as a conduit to deploy a 16 mm 10 mm 7 cm conical Excluder endoprosthesis (contralateral leg of abdominal bifurcated graft, W. L. Gore and Associates). The graft was landed proximally at the origin of the L common carotid artery graft and distally in the common carotid artery (Fig. 2). A small proximal endoleak was seen on postdeployment angiogram and was treated with a second more proximally placed endograft of the same dimensions. The endoleak appeared to be treated, and the decision was made, with the cardiac surgeon, not to convert to open repair. The vertebral artery, thyrocervical trunk, and internal mammary artery were then ligated. The distal end of the Hemashield conduit was anastomosed to the distal subclavian artery in an end-to-end fashion with fenestration of the dissection. The subclavian artery was ligated distal to the aneurysm. The patient had a palpable R radial pulse on completion of the procedure. A postoperative CTA demonstrated complete reconstruction (Fig. 3). A significant proximal type I endoleak was seen on the R (Fig. 4). The patient was presented
Vol. 24, No. 1, January 2010
Fig. 2. Intraoperative angiogram taken during treatment of the R aneurysm. Arrow indicates the location of the R common carotid Hemashield conduit. Inset: Arrow depicts the stent graft being positioned at the distal origin of the L common carotid artery.
with several treatment options and, due to his age and fitness, chose open repair. In conjunction with the cardiothoracic surgeons, the patient underwent redo sternotomy approximately 1 month later. A bypass was created from his aortic arch to the common carotid artery with an 8 mm Dacron graft. The innominate graft was ligated just distal to the take-off of the L common carotid artery. The aneurysm sac was opened, and no back-bleeding was seen. The stent graft was removed, and the subclavian artery origin was oversewn. The patient recovered uneventfully and was discharged home in 6 days. A completion CTA 1 month later showed complete exclusion of both aneurysms without endoleak. The R carotid subclavian artery bypass, L carotid vertebral artery bypass, and aorta R carotid artery bypass were all patent (Fig. 5). The patient will receive the next CTA at 6 months. His follow-up imaging schedule will be based on the results of his CTA at 6 months. The patient is on lifelong coumadin for his mechanical heart valve.
DISCUSSION Subclavian artery aneurysms are uncommon and usually related to atherosclerosis, thoracic outlet syndrome, or trauma.8 Only case reports of
Case reports 114.e3
Fig. 3. Three dimensionally reconstructed CTA after hybrid repair of R and L subclavian artery aneurysms. Arrow shows the patent L carotid to vertebral artery bypass.
subclavian artery aneurysms related to Marfan syndrome exist in the literature4-6,9,10 Patients can present with ischemic complications related to thrombosis or embolization. Rupture is rare, being seen in only two of 31 patients in the series of Pairolero et al.8 Compression-related symptoms can include upper extremity motor and sensory deficits, respiratory distress, hoarseness, Horner syndrome, and dysphagia. Patients commonly complain of upper extremity pain on the affected side.8 A palpable supraclavicular mass and supraclavicular bruit may be found on exam. Our patient was asymptomatic, and his aneurysms were diagnosed on routine follow-up CT scans. Traditional treatment of subclavian artery aneurysms involves sternotomy or thoracotomy with aneurysm resection and restoration of upper extremity perfusion with synthetic or autogenous interposition graft. Long-term patency is excellent; however, morbidity rates can be as high as 46%.8,11 The chance of morbidity may be even higher in Marfan patients due to their propensity for anastomotic breakdown and the possibility of redo aortic surgery. May et al.12 first described exclusion of a subclavian aneurysm with endovascular stent graft. As stent graft and endovascular technology have advanced, the use of stent grafts to treat subclavian aneurysms has become first-line therapy in anatomically appropriate patients due to the lower
114.e4 Case reports
Fig. 4. Axial slice of CTA depicting proximal R innominate portion of stent graft. Arrow indicates an endoleak originating from the proximal seal zone. No evidence of type II endoleak was seen.
morbidity and adequate patency.4,6,13 This technology is even more attractive in patients with connective tissue disorders as it can diminish the need for arterial anastomoses and help avoid reoperative surgery.5 Although endovascular repair has become common, long-term data regarding durability, patency, and maintenance of structure and position in a mobile area are absent. Patients will need more frequent postprocedure imaging studies to ensure adequacy of the repair. Several factors were taken into account when planning this reconstruction. The patient had a prior sternotomy and L thoracotomy, his neo-aortic arch anatomy precluded percutaneous transfemoral treatment, both vertebral artery origins were involved in the aneurysms, the majority of his aorta had been replaced (increasing the risk of spinal cord ischemia with vertebral artery ligation), and no proximal R subclavian landing zone. Also, dissection in the R subclavian artery prevented wire access. Hybrid repair with current endovascular and open technology allowed treatment of the aneurysms with minimal morbidity. This case also highlights some limits of endovascular repair. Despite appropriate sizing and maximal proximal placement of the R-sided stent graft, a type I endoleak was still present. This may have been due to anatomical factors related to the innominate artery anastomosis from the prior aortic arch replacement. Other interventions, such as extension with a bare metal stent covering the L common carotid or coil embolization of the tract, could have
Annals of Vascular Surgery
Fig. 5. Three dimensionally reconstructed CTA of final reconstruction. Arrow indicates a patent aorta to R common carotid artery bypass. Both aneurysms are excluded, with no evidence of endoleak.
been attempted; but a type I endoleak in a young patient requires definitive treatment. Fortunately, this patient was able to tolerate a redo sternotomy without significant morbidity. The combination of current endovascular technology and traditional surgical technique was used to successfully treat this patient with Marfan syndrome and bilateral subclavian artery aneurysms. Endovascular therapy is promising due to the potential for low morbidity. Stent-graft repair in patients with connective tissue disorders may be less durable over time due to arterial dilations at anchor zones. Frequent long-term surveillance is mandatory. REFERENCES 1. Lawrence P, Gazak C, Bhirangi L, et al. The epidemiology of surgically repaired aneurysms in the United States. J Vasc Surg 1999;30:632 640. 2. Scher L, Veith F, Samson R, et al. Vascular complications of thoracic outlet syndrome. J Vasc Surg 1986;3:565 568. 3. Dougherty M, Calligaro K, Savarese R, et al. Atherosclerotic aneurysm of the intrathoracic subclavian artery: a case report and review of the literature. J Vasc Surg 1995;21: 521 529. 4. Kasirajan K, Matteson B, Marek J, et al. Covered stents for true subclavian aneurysm in patients with degenerative connective tissure disorders. J Endovasc Ther 2003;10: 647 652. 5. Gonzalez J, Garcia B, Lebrun J, et al. Combined surgery for the treatment of bilateral subclavian artery aneurysm in Marfan syndrome. J Vasc Surg 2007;45:180 182.
Vol. 24, No. 1, January 2010
6. Tassiopoulos A, Nadalin G, Labropoulos N, et al. Endovascular repair of a symptomatic subclavian artery aneruysm in a patient with Marfan syndrome. J Vasc Endovasc Surg 2006;40:409 413. 7. Etz C, Plestis K, Kari F, et al. Staged repair of thoracic and thoracoabdominal aortic aneurysms using the elephant trunk technique: a consecutive series of 215 first stage and 120 complete repairs. Eur J Cardthorac Surg 2008;34:605 615. 8. Pairolero P, Walks J, Rayne W, et al. Subclavian axillary artery aneurysms. Surgery 1981;90:757 763. 9. Nawa S, Ikeda E, Ichihara S, et al. A true aneurysm of axillarye subcalvian artery with cystic medionecrosis: an unusual min festation of Marfan syndrome. Ann Vasc Surg 2003;17: 562 564.
Case reports 114.e5
10. de Virgilio C, Cherry K, Schaff H. Multiple aneurysms and aortic dissection: an unusual manifestation of Marfan’s syndrome. Ann Vasc Surg 1994;8:383 386. 11. Salo J, Ala Kulju K, Heikkinen L, et al. Diagnosis and treatment of subclavian artery aneurysms. Eur J Vasc Surg 1990;4:271 274. 12. May J, White G, Waugh R, et al. Transluminal placement of a prosthetic graft stent device for treatment of subclavian artery aneurysm. J Vasc Surg 1993;18:1056 1059. 13. Kasirajan K, Matteson B, Marek J, et al. Covered stents for true subclavian aneurysms in patients with degenerative connective tissue disorders. J Endovasc Ther 2003;10: 647 652.