- Email: [email protected]
Endovascular Repair of a Right-sided Thoracic Aortic Aneurysm with Kommerell Diverticulum and Aberrant Left Subclavian Artery
Case Report Endovascular Repair of a Right-sided Thoracic Aortic Aneurysm with Kommerell Diverticulum and Aberrant Left Subclavian Artery Mario Monaco,1 Stefano Lillo,1 Agostino La Marca Giordano,1 Antonio Contaldo,1 and Vincenzo Schiavone,2 Castelvolturno (CE), Italy
Background: A case of successful total endovascular repair of a right-sided descending thoracic aorta aneurysm (r-DTAA) with Kommerell diverticulum and aberrant left subclavian artery (ALSA) was reported. Few cases of this very rare pathology were reported, mostly describing a hybrid treatment, with only 2 cases of total endovascular repair performed to date. Methods and Results: Our strategy consisted of endovascular ALSA occlusion, without preventive revascularization, and r-DTAA exclusion by 2 endoprosthesis implanted in a telescopic fashion, first the distal one, to achieve a relative straightening of the arch and support the proximal endoprosthesis, and then the proximal one, close to the right subclavian origin. Completion angiography and 12-month computed tomography scan showed successful exclusion, patency of epiaortic vessels, and absence of endoleak. Conclusion: Endovascular repair can be a safe and effective treatment for aortic disease with challenging anatomy, avoiding the need for a complex open surgery procedure.
Right-sided aortic arch is a rare pathology1 and even rarer is the presence of a right descending thoracic aorta aneurysm (r-DTAA) and a Kommerell diverticulum (KD) from which arise an aberrant left subclavian artery (ALSA). The incidence of such pathology has been previously reported2,3 and surgical open treatment was indicated for the management of this rare pathology.4e7 More recently, thoracic aortic endovascular repair (TEVAR) associated with aortic arch debranching or surgical 1 Department of Vascular Surgery, Pineta Grande Hospital, Castelvolturno (CE), Italy. 2 Department of Anesthesiology, Pineta Grande Hospital, Castelvolturno (CE), Italy.
Correspondence to: Mario Monaco, MD, Departments of Vascular Surgery and Anesthesiology, Pineta Grande Hospital, via A. Falcone 258, Naples 80127, Italy; E-mail: [email protected] Ann Vasc Surg 2014; -: 1–5 http://dx.doi.org/10.1016/j.avsg.2014.01.023 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: February 20, 2013; manuscript accepted: January 12, 2014; published online: ---.
subclavian revascularization8e12 was employed in few cases. To date, a total endovascular procedure was employed only in 2 cases.13,14 Aortic morphology is often challenging for TEVAR in these cases because of aortic arch tortuousness that could make difficult the endoprosthesis deployment. Otherwise, TEVAR is an appealing treatment for such complex pathology, avoiding a deeply invasive procedure or life-threatening open surgery procedure. We herein report a case of successful total endovascular repair of r-DTAA and concomitant large KD, with endovascular occlusion of ALSA, without preventive revascularization.
CASE REPORT A 76-year-old man was admitted to our department because of an abnormal shadow in the upper mediastinum, suggestive of a thoracic aortic aneurysm. The patient had many severe comorbidities, such as previous coronary artery disease treated with myocardial revascularization, mild chronic renal insufficiency (glomerular 1
2 Case Report
Annals of Vascular Surgery
Fig. 1. (A) Axial CT-scan showing transversal diameters (dotted line, 67 mm; solid line, 56 mm). (B) Sagittal multiplanar reconstruction at the same level. The white arrow shows the aberrant left subclavian artery arising from the Kommerell diverticulum. (C) Intraoperative angiography showing the first stent graft deployed distally, with persistence of Kommerell diverticulum (K). The white arrow indicates the occluder device previously implanted, with complete exclusion of the aberrant left
subclavian artery. (D) Intraoperative angiography showing the second stent graft advanced in the aortic arch through the first stent graft. The white arrow indicates the occluder device previously implanted, with complete exclusion of the aberrant left subclavian artery and persistence of Kommerell diverticulum (K). LC, left common carotid; RC, right common carotid; RS, right subclavian.
filtration rate ¼ 65 mL/m/1.73 m2), and severe chronic obstructive pulmonary disease (forced expiratory volume in the 1th second, % predicted ¼ 45). The patient was classified as high risk according to the American Society of Anesthesiology class IV. Computed tomography (CT) (Fig. 1A) showed an r-DTAA with a saccular aneurysm involving the distal arch and the proximal descending aorta, including a large KD, of 6.7 cm in diameter, with an ALSA arising from the diverticulum. The left and right carotid arteries had originated from the ascending aorta and the right subclavian artery originated from the posterior aspect of the aortic arch, between the left carotid artery and diverticulum (Fig. 1B). Preoperative ultrasonography of epiaortic vessels showed normal morphology and flow of carotid and vertebral arteries. Because of preoperative
high risk, an endovascular aortic repair was planned. The procedure was performed under local anesthesia with full hemodynamic monitoring, including invasive right radial artery pressure, in a dedicated hybrid operative room equipped with a portable imaging system (OEC 9900 Elite; GE Healthcare, General Electric Company, Fairfield, CT). Cerebrospinal fluid drainage (CSFD) was not used perioperatively. Patients underwent anticoagulation with heparin (100 U/kg) with a target activated clotting time of >300 sec during the procedure. Intraoperative aortic angiography confirmed aortic anatomy and normal patency of carotid, vertebral, and cerebral arteries, with codominant vertebral circulation and the basilar artery normally and equally fed from both vertebrals. Therefore direct coverage of the ALSA was planned. Through a
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Case Report 3
Fig. 2. (A) Completion angiography showing final deployment of the 2 stent graft, normal patency of epiaortic vessels, with complete exclusion of Kommerell diverticulum and aberrant LS artery. The white arrow indicates the occluder device previously implanted. (B) Axial CT-scan showing the epiaortic vessels, with normal perfusion in the RC, (RS), and LC arteries. The occluder device is evident in the LS artery with clearly absence
of enhancement. (C) Three-dimensional reconstruction with volume rendering shows the overlapping of the 2 stent grafts (white arrow), the occluder device in the LS, with normal patency of the other epiaortic vessels, without any sign of endoleak. LC, left common carotid; LS, left subclavian; RC, right common carotid; RS, right subclavian.
small incision of the left brachial artery, a 12-mm occluder device (Medtronic AVE, Santa Rosa, CA) was deployed in the ALSA just below the origin of the left vertebral artery, to avoid type II endoleak, without preventive revascularization. Through surgical exposition of the right common femoral artery, a 0.035-inch ultra-stiff guidewire (Backup Meier; Boston Scientific, Miami, FL) was positioned in the ascending aorta. Controlled hypotension was induced by nitroglycerin infusion at the starting rate of 1 mg/kg/min and then adjusted to maintain a 90 mmHg systolic pressure. The proximal neck just below the origin of the right subclavian artery measured 33 mm and the descending aorta below the diverticulum reached a proper diameter (32 mm) just before the first 90 angle of the thoracic aorta (Fig. 1B). Because of a fairly tortuous course of the aortic arch with an aneurismal total length of 20 cm and considering the usual in vivo shortening of stent grafts when deployed in the aortic arch (we consider it to be about 20%) and about 5 cm of overlapping between the 2 stent grafts, we decided to use a 200-cm stent graft first and then a 100-cm stent graft to be sure to cover the entire diseased aortic segment. We planned to first implant the distal endoprosthesis to achieve a relative straightening of the arch and support the proximal one implanted close to the right subclavian origin (Fig. 1C). Over the guidewire, a 36e36 200-mm Valiant endoprosthesis stent graft (Medtronic AVE) was deployed from the distal neck to the middle of the proximal neck and then a 36e 36 100 mm Valiant endoprosthesis stent graft was
advanced in the aortic arch (Fig. 1D) overlapping the right subclavian ostium, the most proximal artery to KD, and then carefully pulled back until optimal pressure curve was reassessed in the right radial artery together with an exact position at the fluoroscopic imaging. Completion angiography showed successful exclusion of the aneurysm, normal patency of both carotid and the right subclavian arteries, and the complete exclusion of the ALSA, without evidence of any type of endoleak (Fig. 2A). Postoperative course was uneventful and the patient was discharged home on postoperative day 4. Three- and 12-month CT-scans have shown no evidence of endoleak (Fig. 2B, C), with normal perfusion of both carotid and the right subclavian arteries.
DISCUSSION The persistence of a remnant of the right dorsal aorta with an aberrant right subclavian artery originating from the descending thoracic aorta of a left-sided arch was first described by Kommerell in 1936. The right aortic arch with ALSA alone as an isolated anomaly is extremely rare. It results from regression of the left fourth aortic arch between the left common carotid and the left subclavian arteries. In these patients, a large aortic diverticulum is often present.1,2
4 Case Report
Our patient had an aneurysm of the distal aortic arch and the initial portion of the r-DTA, involving the KD from which arose the ALSA. TEVAR provides favorable outcomes with decreased morbidity and mortality relative to open repair, mostly in high-risk and emergent patients.15,16 In our patient, TEVAR was indicated because of the presence of much relevant comorbidities, although the challenging anatomy could make it difficult to apply this method. Two of the 3 major prerequisites for TEVAR (sufficient size of the arterial access, limited tortuosity of the aortic arch, and neck morphology) were present in our patient. Indeed, the aortic arch had a fairly tortuous course, but we considered that the relatively straight portions of the aorta from the end of KD to the origin of the right subclavian artery made the landing zone long enough to develop a stent graft. Furthermore, we adopted, in this case, an endovascular technique to overwhelm the difficulty because of the arch tortuousness: the deployment of the 2 endoprosthesis from distal to the proximal landing zone lead to smooth the arch tortuosity, achieving more support to the proximal stent graft for a meticulously precise deployment. Meticulous preoperative imaging can improve diagnostic accuracy and facilitate surgical planning. The presence of an ALSA requires proximal ligation, coil embolization, or occlusion to prevent a type II endoleak. Endovascular deployment of an occluder was a simple and safe method, not precluding selective subclavian revascularization. In our case, the preventive occlusion of the ALSA to prevent a type II endoleak was planned, without elective revascularization, and a high-profile device was used because of KD and ALSA diameter, which we considered too large for a low-profile device use, such as coil or plug.13 LSA coverage is often necessary to achieve proximal seal in up to 40% of patients treated with TEVAR. The management of the LSA in this cohort of patients remains controversial.17,18 In the presence of normal anatomy of epiaortic and cerebral vessels, we agree with selective LSA revascularization during TEVAR (i.e., in those patients with patent left internal mammary artery for coronary bypass, dominant or isolated left vertebral artery, or a functioning left upper extremity dialysis arteriovenous fistula). Similar to open repair, TEVAR carries a risk of spinal cord ischemia (SCI). Although SCI is uncommon after TEVAR,19 CSFD, which may increase the perfusion pressure to the spinal cord, was advocated to prevent such dreadful complications. The role of prophylactic CSFD was not established from the
Annals of Vascular Surgery
available literature in thoracic and thoracoabdominal aneurysm surgery for prevention of neurological injury.19,20 We do not use CSFD routinely during TEVAR, unless more than one risk factor was present (LSA coverage, extensive aortic coverage, occlusion of hypogastric arteries, previous aortic surgery) and we considered in our case the coverage of the thoracic aorta not extensive, because a long segment of thoracic aorta between the end of the stent grafts and the origin of the celiac trunk (Fig. 2C) was not covered, including the critical T8 to L2 segment. We consider actually as extensive aortic coverage, at significant risk of CSI, the coverage extended to within 5 cm of the celiac trunk origin (Fig. 2C), using in these cases preventive CSFD.21 To our knowledge, only few cases of TEVAR of this type of pathology were reported. Okada8 first demonstrated the feasibility of endovascular repair in a patient with an aneurysm involving a rightsided aortic arch and a left-sided descending aorta, with a totally occluded LSA. Other authors, performing mostly hybrid procedures9e12 and only in 2 cases a total endovascular procedure,13,14 confirmed that TEVAR is a viable and safe alternative to open surgical repair in these rare and complex cases.
CONCLUSIONS Our case was a total endovascular repair of an r-DTA aneurysm. Endovascular treatment of right-sided aortic arch aneurysms can be made safely and effectively, although considerable technical dexterity and center experience were required. It is appealing because it obviates the need for a complex open surgery procedure, avoiding a thoracotomy or median sternotomy with the use of cerebral perfusion or circulatory arrest. REFERENCES 1. Hastrier AR, D’Cruz IA. Right-sided aorta. Part 1. Occurrence of right aortic arch in various types of congenital heart disease. Br Heart J 1965;28:722e5. 2. Cina CS, Althani H, Pasenau J, et al. Kommerell’s diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg 2004;39:131e9. 3. Fisher RG, Whigham CJ, Trinh C. Diverticula of Kommerell and aberrant subclavian arteries complicated by aneurysms. Cardiovasc Intervent Radiol 2005;28:553e60. 4. Cooley DA, Mullins CE, Gooch JB. Aneurysm of right-sided cervical arch: surgical removal and graft replacement. J Thorac Cardiovasc Surg 1976;72:106e8. 5. Caus T, Gaubert JY, Monties JR, et al. Right-sided aortic arch: surgical treatment of an aneurysm arising from a Kommerell’s diverticulum and extending to the descending thoracic aorta with an aberrant left subclavian artery. Cardiovasc Surg 1994;2:110e3.
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6. Robinson BL, Nadolny EM, Entrup MH, et al. Management of right-sided aortic arch aneurysms. Ann Thorac Surg 2001;72:1764e5. 7. Tsukube T, Ataka K, Sakata M, et al. Surgical treatment of an aneurysm in the right aortic arch with aberrant left subclavian artery. Ann Thorac Surg 2001;71:1710e1. 8. Okada K, Sueda T, Orihashi K, et al. Endovascular stentgraft repair for thoracic aortic aneurysm associated with right-sided aortic arch. J Thorac Cardiovasc Surg 2001;122: 185e6. 9. Naoum JJ, Parenti LJ, LeMaire AS, et al. Endovascular repair of a right-sided descending thoracic aortic aneurysm with a right-sided aortic arch and aberrant left subclavian artery. Ann Thorac Surg 2008;85:1074e6. 10. Klonaris C, Avgerinos ED, Katsargyris A, et al. Endovascular repair of a right-sided descending thoracic aortic aneurysm associated with a right aortic arch and a left subclavian artery arising from a Kommerell’s diverticulum. Cardiovasc Intervent Radiol 2009;32:758e61. 11. Frigatti P, Grego F, Deriu GP, et al. Hybrid endovascular treatment of aneurysm degeneration in a rare right-aortic arch anomaly with Kommerell diverticulum. J Vasc Surg 2009;50:903e6. 12. Kawajiri H, Shimizu H, Yoshitake A, et al. Hybrid repair of a Kommerell diverticulum associated with a right aortic arch and a left descending aorta. J Vasc Surg 2012;56: 1727e30. 13. Midorikawa H, Kannno M, Ishikawa K, et al. Endovascular repair of a Kommerell’s diverticulum and aneurysmal
Case Report 5
14.
15.
16.
17.
18.
19.
20.
21.
right-sided aortic arch: a case report. Ann Vasc Dis 2009;2:54e7. Silveira PG, Franklin RN, Cunha JR, et al. Total endovascular repair of aberrant left subclavian artery with Kommerell’s diverticulum using a customized branched device. J Vasc Surg 2013;57:1123e5. Jonker FH, Trimarchi S, Verhagen HJ, et al. Meta-analysis of open versus endovascular repair for ruptured descending thoracic aortic aneurysm. J Vasc Surg 2010;51:1026e32. Gopaldas RR, Dao TK, LeMaire SA, et al. Endovascular versus open repair of ruptured descending thoracic aortic aneurysms: a nationwide risk-adjusted study of 923 patients. J Thorac Cardiovasc Surg 2011;142:1010e8. Dexter D, Maldonado TS. Left subclavian artery coverage during TEVAR: is revascularization necessary? J Cardiovasc Surg 2012;53:135e41. Weigang E, Parker JA, Czerny M, et al. Should intentional endovascular stent-graft coverage of the left subclavian artery be preceded by prophylactic revascularization? Eur J Cardiothorac Surg 2011;40:858e68. Wong CS, Healy D, Canning C, et al. A systematic review of spinal cord injury and cerebrospinal fluid drainage after thoracic aortic endografting. J Vasc Surg 2012;56:1438e47. Khan SN, Stansby G. Cerebrospinal fluid drainage for thoracic and thoracoabdominal aortic aneurysm surgery. Cochrane Database Syst Rev 2012;(10):CD003635. Lee WA, Daniels MJ, Beaver TM, et al. Late outcomes of a single-center experience of 400 consecutive thoracic endovascular aortic repairs. Circulation 2011;123:2938e45.
Background: A case of successful total endovascular repair of a right-sided descending thoracic aorta aneurysm (r-DTAA) with Kommerell diverticulum and aberrant left subclavian artery (ALSA) was reported. Few cases of this very rare pathology were reported, mostly describing a hybrid treatment, with only 2 cases of total endovascular repair performed to date. Methods and Results: Our strategy consisted of endovascular ALSA occlusion, without preventive revascularization, and r-DTAA exclusion by 2 endoprosthesis implanted in a telescopic fashion, first the distal one, to achieve a relative straightening of the arch and support the proximal endoprosthesis, and then the proximal one, close to the right subclavian origin. Completion angiography and 12-month computed tomography scan showed successful exclusion, patency of epiaortic vessels, and absence of endoleak. Conclusion: Endovascular repair can be a safe and effective treatment for aortic disease with challenging anatomy, avoiding the need for a complex open surgery procedure.
Right-sided aortic arch is a rare pathology1 and even rarer is the presence of a right descending thoracic aorta aneurysm (r-DTAA) and a Kommerell diverticulum (KD) from which arise an aberrant left subclavian artery (ALSA). The incidence of such pathology has been previously reported2,3 and surgical open treatment was indicated for the management of this rare pathology.4e7 More recently, thoracic aortic endovascular repair (TEVAR) associated with aortic arch debranching or surgical 1 Department of Vascular Surgery, Pineta Grande Hospital, Castelvolturno (CE), Italy. 2 Department of Anesthesiology, Pineta Grande Hospital, Castelvolturno (CE), Italy.
Correspondence to: Mario Monaco, MD, Departments of Vascular Surgery and Anesthesiology, Pineta Grande Hospital, via A. Falcone 258, Naples 80127, Italy; E-mail: [email protected] Ann Vasc Surg 2014; -: 1–5 http://dx.doi.org/10.1016/j.avsg.2014.01.023 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: February 20, 2013; manuscript accepted: January 12, 2014; published online: ---.
subclavian revascularization8e12 was employed in few cases. To date, a total endovascular procedure was employed only in 2 cases.13,14 Aortic morphology is often challenging for TEVAR in these cases because of aortic arch tortuousness that could make difficult the endoprosthesis deployment. Otherwise, TEVAR is an appealing treatment for such complex pathology, avoiding a deeply invasive procedure or life-threatening open surgery procedure. We herein report a case of successful total endovascular repair of r-DTAA and concomitant large KD, with endovascular occlusion of ALSA, without preventive revascularization.
CASE REPORT A 76-year-old man was admitted to our department because of an abnormal shadow in the upper mediastinum, suggestive of a thoracic aortic aneurysm. The patient had many severe comorbidities, such as previous coronary artery disease treated with myocardial revascularization, mild chronic renal insufficiency (glomerular 1
2 Case Report
Annals of Vascular Surgery
Fig. 1. (A) Axial CT-scan showing transversal diameters (dotted line, 67 mm; solid line, 56 mm). (B) Sagittal multiplanar reconstruction at the same level. The white arrow shows the aberrant left subclavian artery arising from the Kommerell diverticulum. (C) Intraoperative angiography showing the first stent graft deployed distally, with persistence of Kommerell diverticulum (K). The white arrow indicates the occluder device previously implanted, with complete exclusion of the aberrant left
subclavian artery. (D) Intraoperative angiography showing the second stent graft advanced in the aortic arch through the first stent graft. The white arrow indicates the occluder device previously implanted, with complete exclusion of the aberrant left subclavian artery and persistence of Kommerell diverticulum (K). LC, left common carotid; RC, right common carotid; RS, right subclavian.
filtration rate ¼ 65 mL/m/1.73 m2), and severe chronic obstructive pulmonary disease (forced expiratory volume in the 1th second, % predicted ¼ 45). The patient was classified as high risk according to the American Society of Anesthesiology class IV. Computed tomography (CT) (Fig. 1A) showed an r-DTAA with a saccular aneurysm involving the distal arch and the proximal descending aorta, including a large KD, of 6.7 cm in diameter, with an ALSA arising from the diverticulum. The left and right carotid arteries had originated from the ascending aorta and the right subclavian artery originated from the posterior aspect of the aortic arch, between the left carotid artery and diverticulum (Fig. 1B). Preoperative ultrasonography of epiaortic vessels showed normal morphology and flow of carotid and vertebral arteries. Because of preoperative
high risk, an endovascular aortic repair was planned. The procedure was performed under local anesthesia with full hemodynamic monitoring, including invasive right radial artery pressure, in a dedicated hybrid operative room equipped with a portable imaging system (OEC 9900 Elite; GE Healthcare, General Electric Company, Fairfield, CT). Cerebrospinal fluid drainage (CSFD) was not used perioperatively. Patients underwent anticoagulation with heparin (100 U/kg) with a target activated clotting time of >300 sec during the procedure. Intraoperative aortic angiography confirmed aortic anatomy and normal patency of carotid, vertebral, and cerebral arteries, with codominant vertebral circulation and the basilar artery normally and equally fed from both vertebrals. Therefore direct coverage of the ALSA was planned. Through a
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Case Report 3
Fig. 2. (A) Completion angiography showing final deployment of the 2 stent graft, normal patency of epiaortic vessels, with complete exclusion of Kommerell diverticulum and aberrant LS artery. The white arrow indicates the occluder device previously implanted. (B) Axial CT-scan showing the epiaortic vessels, with normal perfusion in the RC, (RS), and LC arteries. The occluder device is evident in the LS artery with clearly absence
of enhancement. (C) Three-dimensional reconstruction with volume rendering shows the overlapping of the 2 stent grafts (white arrow), the occluder device in the LS, with normal patency of the other epiaortic vessels, without any sign of endoleak. LC, left common carotid; LS, left subclavian; RC, right common carotid; RS, right subclavian.
small incision of the left brachial artery, a 12-mm occluder device (Medtronic AVE, Santa Rosa, CA) was deployed in the ALSA just below the origin of the left vertebral artery, to avoid type II endoleak, without preventive revascularization. Through surgical exposition of the right common femoral artery, a 0.035-inch ultra-stiff guidewire (Backup Meier; Boston Scientific, Miami, FL) was positioned in the ascending aorta. Controlled hypotension was induced by nitroglycerin infusion at the starting rate of 1 mg/kg/min and then adjusted to maintain a 90 mmHg systolic pressure. The proximal neck just below the origin of the right subclavian artery measured 33 mm and the descending aorta below the diverticulum reached a proper diameter (32 mm) just before the first 90 angle of the thoracic aorta (Fig. 1B). Because of a fairly tortuous course of the aortic arch with an aneurismal total length of 20 cm and considering the usual in vivo shortening of stent grafts when deployed in the aortic arch (we consider it to be about 20%) and about 5 cm of overlapping between the 2 stent grafts, we decided to use a 200-cm stent graft first and then a 100-cm stent graft to be sure to cover the entire diseased aortic segment. We planned to first implant the distal endoprosthesis to achieve a relative straightening of the arch and support the proximal one implanted close to the right subclavian origin (Fig. 1C). Over the guidewire, a 36e36 200-mm Valiant endoprosthesis stent graft (Medtronic AVE) was deployed from the distal neck to the middle of the proximal neck and then a 36e 36 100 mm Valiant endoprosthesis stent graft was
advanced in the aortic arch (Fig. 1D) overlapping the right subclavian ostium, the most proximal artery to KD, and then carefully pulled back until optimal pressure curve was reassessed in the right radial artery together with an exact position at the fluoroscopic imaging. Completion angiography showed successful exclusion of the aneurysm, normal patency of both carotid and the right subclavian arteries, and the complete exclusion of the ALSA, without evidence of any type of endoleak (Fig. 2A). Postoperative course was uneventful and the patient was discharged home on postoperative day 4. Three- and 12-month CT-scans have shown no evidence of endoleak (Fig. 2B, C), with normal perfusion of both carotid and the right subclavian arteries.
DISCUSSION The persistence of a remnant of the right dorsal aorta with an aberrant right subclavian artery originating from the descending thoracic aorta of a left-sided arch was first described by Kommerell in 1936. The right aortic arch with ALSA alone as an isolated anomaly is extremely rare. It results from regression of the left fourth aortic arch between the left common carotid and the left subclavian arteries. In these patients, a large aortic diverticulum is often present.1,2
4 Case Report
Our patient had an aneurysm of the distal aortic arch and the initial portion of the r-DTA, involving the KD from which arose the ALSA. TEVAR provides favorable outcomes with decreased morbidity and mortality relative to open repair, mostly in high-risk and emergent patients.15,16 In our patient, TEVAR was indicated because of the presence of much relevant comorbidities, although the challenging anatomy could make it difficult to apply this method. Two of the 3 major prerequisites for TEVAR (sufficient size of the arterial access, limited tortuosity of the aortic arch, and neck morphology) were present in our patient. Indeed, the aortic arch had a fairly tortuous course, but we considered that the relatively straight portions of the aorta from the end of KD to the origin of the right subclavian artery made the landing zone long enough to develop a stent graft. Furthermore, we adopted, in this case, an endovascular technique to overwhelm the difficulty because of the arch tortuousness: the deployment of the 2 endoprosthesis from distal to the proximal landing zone lead to smooth the arch tortuosity, achieving more support to the proximal stent graft for a meticulously precise deployment. Meticulous preoperative imaging can improve diagnostic accuracy and facilitate surgical planning. The presence of an ALSA requires proximal ligation, coil embolization, or occlusion to prevent a type II endoleak. Endovascular deployment of an occluder was a simple and safe method, not precluding selective subclavian revascularization. In our case, the preventive occlusion of the ALSA to prevent a type II endoleak was planned, without elective revascularization, and a high-profile device was used because of KD and ALSA diameter, which we considered too large for a low-profile device use, such as coil or plug.13 LSA coverage is often necessary to achieve proximal seal in up to 40% of patients treated with TEVAR. The management of the LSA in this cohort of patients remains controversial.17,18 In the presence of normal anatomy of epiaortic and cerebral vessels, we agree with selective LSA revascularization during TEVAR (i.e., in those patients with patent left internal mammary artery for coronary bypass, dominant or isolated left vertebral artery, or a functioning left upper extremity dialysis arteriovenous fistula). Similar to open repair, TEVAR carries a risk of spinal cord ischemia (SCI). Although SCI is uncommon after TEVAR,19 CSFD, which may increase the perfusion pressure to the spinal cord, was advocated to prevent such dreadful complications. The role of prophylactic CSFD was not established from the
Annals of Vascular Surgery
available literature in thoracic and thoracoabdominal aneurysm surgery for prevention of neurological injury.19,20 We do not use CSFD routinely during TEVAR, unless more than one risk factor was present (LSA coverage, extensive aortic coverage, occlusion of hypogastric arteries, previous aortic surgery) and we considered in our case the coverage of the thoracic aorta not extensive, because a long segment of thoracic aorta between the end of the stent grafts and the origin of the celiac trunk (Fig. 2C) was not covered, including the critical T8 to L2 segment. We consider actually as extensive aortic coverage, at significant risk of CSI, the coverage extended to within 5 cm of the celiac trunk origin (Fig. 2C), using in these cases preventive CSFD.21 To our knowledge, only few cases of TEVAR of this type of pathology were reported. Okada8 first demonstrated the feasibility of endovascular repair in a patient with an aneurysm involving a rightsided aortic arch and a left-sided descending aorta, with a totally occluded LSA. Other authors, performing mostly hybrid procedures9e12 and only in 2 cases a total endovascular procedure,13,14 confirmed that TEVAR is a viable and safe alternative to open surgical repair in these rare and complex cases.
CONCLUSIONS Our case was a total endovascular repair of an r-DTA aneurysm. Endovascular treatment of right-sided aortic arch aneurysms can be made safely and effectively, although considerable technical dexterity and center experience were required. It is appealing because it obviates the need for a complex open surgery procedure, avoiding a thoracotomy or median sternotomy with the use of cerebral perfusion or circulatory arrest. REFERENCES 1. Hastrier AR, D’Cruz IA. Right-sided aorta. Part 1. Occurrence of right aortic arch in various types of congenital heart disease. Br Heart J 1965;28:722e5. 2. Cina CS, Althani H, Pasenau J, et al. Kommerell’s diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg 2004;39:131e9. 3. Fisher RG, Whigham CJ, Trinh C. Diverticula of Kommerell and aberrant subclavian arteries complicated by aneurysms. Cardiovasc Intervent Radiol 2005;28:553e60. 4. Cooley DA, Mullins CE, Gooch JB. Aneurysm of right-sided cervical arch: surgical removal and graft replacement. J Thorac Cardiovasc Surg 1976;72:106e8. 5. Caus T, Gaubert JY, Monties JR, et al. Right-sided aortic arch: surgical treatment of an aneurysm arising from a Kommerell’s diverticulum and extending to the descending thoracic aorta with an aberrant left subclavian artery. Cardiovasc Surg 1994;2:110e3.
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6. Robinson BL, Nadolny EM, Entrup MH, et al. Management of right-sided aortic arch aneurysms. Ann Thorac Surg 2001;72:1764e5. 7. Tsukube T, Ataka K, Sakata M, et al. Surgical treatment of an aneurysm in the right aortic arch with aberrant left subclavian artery. Ann Thorac Surg 2001;71:1710e1. 8. Okada K, Sueda T, Orihashi K, et al. Endovascular stentgraft repair for thoracic aortic aneurysm associated with right-sided aortic arch. J Thorac Cardiovasc Surg 2001;122: 185e6. 9. Naoum JJ, Parenti LJ, LeMaire AS, et al. Endovascular repair of a right-sided descending thoracic aortic aneurysm with a right-sided aortic arch and aberrant left subclavian artery. Ann Thorac Surg 2008;85:1074e6. 10. Klonaris C, Avgerinos ED, Katsargyris A, et al. Endovascular repair of a right-sided descending thoracic aortic aneurysm associated with a right aortic arch and a left subclavian artery arising from a Kommerell’s diverticulum. Cardiovasc Intervent Radiol 2009;32:758e61. 11. Frigatti P, Grego F, Deriu GP, et al. Hybrid endovascular treatment of aneurysm degeneration in a rare right-aortic arch anomaly with Kommerell diverticulum. J Vasc Surg 2009;50:903e6. 12. Kawajiri H, Shimizu H, Yoshitake A, et al. Hybrid repair of a Kommerell diverticulum associated with a right aortic arch and a left descending aorta. J Vasc Surg 2012;56: 1727e30. 13. Midorikawa H, Kannno M, Ishikawa K, et al. Endovascular repair of a Kommerell’s diverticulum and aneurysmal
Case Report 5
14.
15.
16.
17.
18.
19.
20.
21.
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