- Email: [email protected]
Multiple Penetrating Aortic Ulcers Involving the Aortic Arch and Brachiocephalic Artery
Ann Thorac Surg 2010;90:997–9
Comment The mitral mass is a rare complication of Wegener’s granulomatosis and has only been reported once in the literature [5]. Cardiac lesions in Wegener’s disease are benign, but they are progressive and can result in the destruction of cardiac structures. Despite the rarity of mitral valve involvement, we believe that patients with Wegener’s disease should undergo serial echocardiography to identify and monitor valvular involvement and consider an operation at an early stage. In addition, we believe that use of three-dimensional echocardiogram, magnetic resonance imaging, or any other three-dimensional modalities should be considered to assess the extent of the resection required.
References 1. Hoffinan GS, Kerr GS, Leavitt RY, et al. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116: 488 –98. 2. Pinching AJ, Lockwood CM, Pussell BA, et al. Wegener’s granulomatosis: observations on 18 patients with severe renal disease. Q J Med 1983;208:435– 60. 3. Grant SC, Levy RD, Venning MC, Ward C, Brooks NH. Wegener’s granulomatosis and the heart. Br Heart J 1994;71: 82– 6. 4. Forstot JZ, Overlie PA, Neufeld GK, Harmon CE, Forstot SL. Cardiac complications of Wegener granulomatosis: a case report of complete heart block and review of the literature. Semin Arthritis Rheum 1980;10:148 –54. 5. Herbst A, Padilla MT, Prasad AR, Morales MC, Copeland JG. Cardiac Wegener’s granulomatosis masquerading as left atrial myxoma. Ann Thorac Surg 2003;75:1321–3. © 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
997
Multiple Penetrating Aortic Ulcers Involving the Aortic Arch and Brachiocephalic Artery Benjamin Hickey, MRCS, Paul Vaughan, MRCS Ed, Allan Dawson, FRCPath, and Aprim Youhana, FRCS(C-Th) Departments of Cardiothoracic Surgery and Pathology, Morriston Hospital, Swansea, Wales, United Kingdom
Penetrating aortic ulcers are frequently observed in the descending thoracic and abdominal aorta. They are uncommon in the aortic arch, and they are extremely rare in the major branches of the aorta. We present the case of a 71-year-old woman who presented with multiple penetrating aortic ulcers affecting both the aortic arch and the brachiocephalic artery, and its successful treatment. (Ann Thorac Surg 2010;90:997–9) © 2010 by The Society of Thoracic Surgeons
P
enetrating atherosclerotic ulcers are generally regarded as part of the spectrum of conditions that constitute the acute aortic syndromes. Their cause and natural history are poorly understood and thus management is controversial. They are known to affect the descending thoracic aorta most frequently in elderly hypertensive patients. Lesions affecting the ascending aorta and arch are less common, and involvement of major aortic branches is extremely rare. A 71-year-old woman (an ex-smoker) was referred to our institution after an incidental finding of an abnormal aortic knuckle on chest roentgenogram. She had symptoms of upper backache for the last 2 years. Apart from controlled hypertension (blood pressure, 130/80 mm Hg at clinic review), her previous medical history and clinical examination were unremarkable. A contrast-enhanced computed tomographic scan of the thorax and abdomen revealed a severely atheromatous distal ascending arch and descending thoracic aorta with a maximum diameter in the arch of 29.4 mm. The scan also revealed several penetrating ulcers within the distal ascending aorta and aortic arch, located adjacent to the origin of the left subclavian artery and close to the origin of the brachiocephalic artery (Figs 1 and 2). A transthoracic echocardiography demonstrated good left ventricular function without valvular pathology, and a coronary angiography was similarly unremarkable. Syphilis serology and autoimmune profiles were all negative. After institution of cardiopulmonary bypass, the ascending aorta, aortic arch, and head and neck vessels
Accepted for publication Feb 9, 2010. Address correspondence to Dr Vaughan, Department of Cardiothoracic Surgery, Morriston Hospital, Heol Maes Eglwys, Swansea, Wales, SA6 6NL, United Kingdom; e-mail: [email protected].
0003-4975/$36.00 doi:10.1016/j.athoracsur.2010.02.050
FEATURE ARTICLES
by the same fibrous material as the mitral valve. This fibrous pillar was partially occluding the left ventricular outflow. The aorta was then opened and the aortic valve was assessed. The noncoronary leaflet of the aortic valve was 4-mm to 5-mm thick and was part of the same process that had replaced the anterior mitral leaflet. In view of this rigid cusp, the aortic valve was explanted and most of the aorto-mitral curtain was excised to debulk the mass, which in part was friable, but with areas of calcification. We replaced the valves with bioprostheses; the mitral valve was replaced with a size 31-mm St. Jude Epic (St. Jude Medical Inc, Minneapolis, MN) and the aortic valve was replaced with a size 2-mm Elan stentless valve (Bellshill, Scotland, United Kingdom). The choice of a tissue stentless valve in the aortic position was on the basis of a nonrigid implant with a good effective orifice area into very fragile tissues. The mitral bioprosthesis was chosen for its flexible cuff. The patient made a full recovery postoperatively. Histology of the lesion showed features of acute and chronic inflammation, myxoid degeneration, prominent eosinophilic infiltration, and some small blood vessels showing intimal fibrosis, which confirmed to be fully consistent with Wegner’s disease.
CASE REPORT HICKEY ET AL MULTIPLE PENETRATING AORTIC ULCERS
998
CASE REPORT HICKEY ET AL MULTIPLE PENETRATING AORTIC ULCERS
Ann Thorac Surg 2010;90:997–9
brachiocephalic trunk; the left common carotid and left subclavian arteries were mobilized on a small island of aorta. The aortic arch was replaced with a size 28-mm Intergard graft (W. L. Gore & Assoc) anastomosed to the free edges of the proximal descending aorta, just distal to the aortic isthmus. The left common carotid and subclavian artery island was anastomosed to the top of the neo-arch. The brachiocephalic trunk was then anastomosed to the neo-arch with a 14-mm Intergard interposition graft. Postoperatively, the patient was transferred to intensive care unit, extubated on the first postoperative day, and discharged home on postoperative day 6, having made an uneventful in-hospital recovery. She remains well at follow-up 6 months postoperatively, and will continue to have aortic surveillance. Histologic examination of the specimen revealed imminent rupture of the aortic wall (Fig 4). Within the ulceration, focal areas of intimal and medial necrosis were evident with occasional giant cells, suggestive of inflammation. These were completely separate from the areas of severe atherosclerosis. In keeping with the necrosis, there was destruction of the normal elastic fibre architecture within the media without surrounding fibrosis. There were no specific features, however, to suggest the cause of these lesions.
FEATURE ARTICLES
Comment Penetrating atherosclerotic ulcers (PAUs) of the thoracic aorta were first described by Shennan in 1934, although they were only described as a separate clinical entity in 1986 [1], and are now generally regarded as part of the spectrum of conditions that constitute the
Fig 1. Computed tomographic scan reconstruction demonstrating atheromatous aorta and penetrating ulcers (white arrows) affecting the brachiocephalic trunk (left arrow) and zenith of the aortic arch (right arrow).
were fully mobilized. On the surface of the arch of the aorta, four penetrating ulcers were identified: (1) an impending rupture in the mid anterior wall at the zenith of the arch (Fig 3), (2) a large ulcer involving the origin of the brachiocephalic trunk, (3) a smaller ulcer between the left subclavian artery and left common carotid artery, and (4) an ulcer just distal to the left subclavian artery. As previously documented, the distal ascending aorta, arch, and proximal descending aorta were all severely atheromatous. The ascending aorta from the sinotubular junction was excised and replaced with a size 30-mm Intergard tube graft (W. L. Gore & Assoc, Flagstaff, AZ). The aortic arch was excised with the proximal 2 cm of the
Fig 2. Three-dimensional computed tomographic reconstruction demonstrating the atheromatous aorta and penetrating ulcers (*).
Ann Thorac Surg 2010;90:997–9
999
Fig 4. A resected specimen demonstrating severe atheromatous disease and imminent rupture (arrow) of the ulcer adjacent to the left subclavian artery.
sive literature search found only a single reference to PAU involving a major branch of the aorta [6]. The natural history and thus the management of PAUs are currently controversial, mainly because of the variety of clinical presentations, ranging from the asymptomatic incidental lesion to symptomatic aortic rupture and cardiovascular collapse. The site of the lesion should also be taken into consideration, as PAU affecting the ascending aorta and arch are believed to be more at risk of complications, including rupture [3, 5], and thus surgical intervention is advocated even if the lesion is asymptomatic [7]. In summary, we have presented an unusual case of PAU affecting the aortic arch and a major branch of the aorta successfully managed with resection of all the lesions and interposition grafting.
References
Fig 3. Intraoperative photograph demonstrating two penetrating ulcers (*). The left affects the proximal brachiocephalic trunk, and the right affects the zenith of the aortic arch.
1. Stanson AW, Kazamier FJ, Hollier LH, et al. Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinical copathologic correlations. Ann Vasc Surg 1986;1: 15–23. 2. Vilacosta I, San Roman JA. Acute aortic syndrome. Heart 2001;85:365– 8. 3. Troxler M, Mavor AID, Homer-Vanniasinkam S. Br J Surg 2001;88:1169 –77. 4. Cho KR, Stanson AW, Potter DD, Cherry KJ, Schaff HV, Sundt TM 3rd. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch. J Thorac Cardiovasc Surg 2004;127: 1393– 401. 5. Sundt TM. Intramural hematoma and penetrating atherosclerotic ulcer of the aorta. Ann Thorac Surg 2007;83:S835– 41. 6. Chu B, Yuan C, Takaya N, Shewchuck JR, Clowes AW, Hatsukami TS. Serial high-spatial-resolution, multi-sequence magnetic resonance imaging studies identify fibrous cap rupture and penetrating ulcer into carotid atherosclerotic plaque. Circulation 2006;113:e660 –1. 7. Movsowitz HD, Lampert C, Jacobs LE, Kotler MN. Penetrating atherosclerotic aortic ulcers. Am Heart J 1994;128:1210 –7.
FEATURE ARTICLES
acute aortic syndromes [2]. The PAUs are typically seen in elderly patients with hypertension and atherosclerosis [3, 4]. Histologically they are described as an atherosclerotic lesion with ulceration that penetrates the internal elastic lamina and allows hematoma formation within the medial layer of the aortic wall [1]. It is well documented that a PAU can result in an intramural hematoma; however this is not universal, presumably as medial fibrosis secondary to chronic atherosclerosis prevents propagation of the hematoma [5]. Despite the evidence thus far, penetrating ulcers are still poorly characterized. It is unclear if they are related to atherosclerosis or other pathologic mechanisms. In our case, the presence of giant cells suggests an inflammatory component to the medial necrosis. This is further underlined by the fact that the lesions were found to be completely separate to the areas of atherosclerosis. However, the autoimmune profile and syphilis serology were both negative, which may suggest an atherosclerotic process. These findings further accentuate the fact that the cause of the penetrating ulcers is unclear, and whether the inflammatory component is the cause of the penetrating ulcers or secondary to the presence of penetrating ulcers further complicates an already poorly understood phenomenon. The PAUs are frequently observed in the descending thoracic and abdominal aorta; the ulcers are often multiple in number and range in size from 2 to 25 mm diameter and 4 to 30 mm in depth [3]. It is uncommon to find PAUs in the aortic arch. Troxler and colleagues [3] report 18 PAU within the arch from 130 patients affected. A similar review from the Mayo Clinic found PAU affecting the arch in only 9 of 119 patients [4]. The presence of multiple PAU within the arch in our patient could be described as unusual, but the occurrence of an ulcer involving the proximal brachiocephalic trunk makes this case almost unique. An exten-
CASE REPORT HICKEY ET AL MULTIPLE PENETRATING AORTIC ULCERS
Comment The mitral mass is a rare complication of Wegener’s granulomatosis and has only been reported once in the literature [5]. Cardiac lesions in Wegener’s disease are benign, but they are progressive and can result in the destruction of cardiac structures. Despite the rarity of mitral valve involvement, we believe that patients with Wegener’s disease should undergo serial echocardiography to identify and monitor valvular involvement and consider an operation at an early stage. In addition, we believe that use of three-dimensional echocardiogram, magnetic resonance imaging, or any other three-dimensional modalities should be considered to assess the extent of the resection required.
References 1. Hoffinan GS, Kerr GS, Leavitt RY, et al. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116: 488 –98. 2. Pinching AJ, Lockwood CM, Pussell BA, et al. Wegener’s granulomatosis: observations on 18 patients with severe renal disease. Q J Med 1983;208:435– 60. 3. Grant SC, Levy RD, Venning MC, Ward C, Brooks NH. Wegener’s granulomatosis and the heart. Br Heart J 1994;71: 82– 6. 4. Forstot JZ, Overlie PA, Neufeld GK, Harmon CE, Forstot SL. Cardiac complications of Wegener granulomatosis: a case report of complete heart block and review of the literature. Semin Arthritis Rheum 1980;10:148 –54. 5. Herbst A, Padilla MT, Prasad AR, Morales MC, Copeland JG. Cardiac Wegener’s granulomatosis masquerading as left atrial myxoma. Ann Thorac Surg 2003;75:1321–3. © 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
997
Multiple Penetrating Aortic Ulcers Involving the Aortic Arch and Brachiocephalic Artery Benjamin Hickey, MRCS, Paul Vaughan, MRCS Ed, Allan Dawson, FRCPath, and Aprim Youhana, FRCS(C-Th) Departments of Cardiothoracic Surgery and Pathology, Morriston Hospital, Swansea, Wales, United Kingdom
Penetrating aortic ulcers are frequently observed in the descending thoracic and abdominal aorta. They are uncommon in the aortic arch, and they are extremely rare in the major branches of the aorta. We present the case of a 71-year-old woman who presented with multiple penetrating aortic ulcers affecting both the aortic arch and the brachiocephalic artery, and its successful treatment. (Ann Thorac Surg 2010;90:997–9) © 2010 by The Society of Thoracic Surgeons
P
enetrating atherosclerotic ulcers are generally regarded as part of the spectrum of conditions that constitute the acute aortic syndromes. Their cause and natural history are poorly understood and thus management is controversial. They are known to affect the descending thoracic aorta most frequently in elderly hypertensive patients. Lesions affecting the ascending aorta and arch are less common, and involvement of major aortic branches is extremely rare. A 71-year-old woman (an ex-smoker) was referred to our institution after an incidental finding of an abnormal aortic knuckle on chest roentgenogram. She had symptoms of upper backache for the last 2 years. Apart from controlled hypertension (blood pressure, 130/80 mm Hg at clinic review), her previous medical history and clinical examination were unremarkable. A contrast-enhanced computed tomographic scan of the thorax and abdomen revealed a severely atheromatous distal ascending arch and descending thoracic aorta with a maximum diameter in the arch of 29.4 mm. The scan also revealed several penetrating ulcers within the distal ascending aorta and aortic arch, located adjacent to the origin of the left subclavian artery and close to the origin of the brachiocephalic artery (Figs 1 and 2). A transthoracic echocardiography demonstrated good left ventricular function without valvular pathology, and a coronary angiography was similarly unremarkable. Syphilis serology and autoimmune profiles were all negative. After institution of cardiopulmonary bypass, the ascending aorta, aortic arch, and head and neck vessels
Accepted for publication Feb 9, 2010. Address correspondence to Dr Vaughan, Department of Cardiothoracic Surgery, Morriston Hospital, Heol Maes Eglwys, Swansea, Wales, SA6 6NL, United Kingdom; e-mail: [email protected].
0003-4975/$36.00 doi:10.1016/j.athoracsur.2010.02.050
FEATURE ARTICLES
by the same fibrous material as the mitral valve. This fibrous pillar was partially occluding the left ventricular outflow. The aorta was then opened and the aortic valve was assessed. The noncoronary leaflet of the aortic valve was 4-mm to 5-mm thick and was part of the same process that had replaced the anterior mitral leaflet. In view of this rigid cusp, the aortic valve was explanted and most of the aorto-mitral curtain was excised to debulk the mass, which in part was friable, but with areas of calcification. We replaced the valves with bioprostheses; the mitral valve was replaced with a size 31-mm St. Jude Epic (St. Jude Medical Inc, Minneapolis, MN) and the aortic valve was replaced with a size 2-mm Elan stentless valve (Bellshill, Scotland, United Kingdom). The choice of a tissue stentless valve in the aortic position was on the basis of a nonrigid implant with a good effective orifice area into very fragile tissues. The mitral bioprosthesis was chosen for its flexible cuff. The patient made a full recovery postoperatively. Histology of the lesion showed features of acute and chronic inflammation, myxoid degeneration, prominent eosinophilic infiltration, and some small blood vessels showing intimal fibrosis, which confirmed to be fully consistent with Wegner’s disease.
CASE REPORT HICKEY ET AL MULTIPLE PENETRATING AORTIC ULCERS
998
CASE REPORT HICKEY ET AL MULTIPLE PENETRATING AORTIC ULCERS
Ann Thorac Surg 2010;90:997–9
brachiocephalic trunk; the left common carotid and left subclavian arteries were mobilized on a small island of aorta. The aortic arch was replaced with a size 28-mm Intergard graft (W. L. Gore & Assoc) anastomosed to the free edges of the proximal descending aorta, just distal to the aortic isthmus. The left common carotid and subclavian artery island was anastomosed to the top of the neo-arch. The brachiocephalic trunk was then anastomosed to the neo-arch with a 14-mm Intergard interposition graft. Postoperatively, the patient was transferred to intensive care unit, extubated on the first postoperative day, and discharged home on postoperative day 6, having made an uneventful in-hospital recovery. She remains well at follow-up 6 months postoperatively, and will continue to have aortic surveillance. Histologic examination of the specimen revealed imminent rupture of the aortic wall (Fig 4). Within the ulceration, focal areas of intimal and medial necrosis were evident with occasional giant cells, suggestive of inflammation. These were completely separate from the areas of severe atherosclerosis. In keeping with the necrosis, there was destruction of the normal elastic fibre architecture within the media without surrounding fibrosis. There were no specific features, however, to suggest the cause of these lesions.
FEATURE ARTICLES
Comment Penetrating atherosclerotic ulcers (PAUs) of the thoracic aorta were first described by Shennan in 1934, although they were only described as a separate clinical entity in 1986 [1], and are now generally regarded as part of the spectrum of conditions that constitute the
Fig 1. Computed tomographic scan reconstruction demonstrating atheromatous aorta and penetrating ulcers (white arrows) affecting the brachiocephalic trunk (left arrow) and zenith of the aortic arch (right arrow).
were fully mobilized. On the surface of the arch of the aorta, four penetrating ulcers were identified: (1) an impending rupture in the mid anterior wall at the zenith of the arch (Fig 3), (2) a large ulcer involving the origin of the brachiocephalic trunk, (3) a smaller ulcer between the left subclavian artery and left common carotid artery, and (4) an ulcer just distal to the left subclavian artery. As previously documented, the distal ascending aorta, arch, and proximal descending aorta were all severely atheromatous. The ascending aorta from the sinotubular junction was excised and replaced with a size 30-mm Intergard tube graft (W. L. Gore & Assoc, Flagstaff, AZ). The aortic arch was excised with the proximal 2 cm of the
Fig 2. Three-dimensional computed tomographic reconstruction demonstrating the atheromatous aorta and penetrating ulcers (*).
Ann Thorac Surg 2010;90:997–9
999
Fig 4. A resected specimen demonstrating severe atheromatous disease and imminent rupture (arrow) of the ulcer adjacent to the left subclavian artery.
sive literature search found only a single reference to PAU involving a major branch of the aorta [6]. The natural history and thus the management of PAUs are currently controversial, mainly because of the variety of clinical presentations, ranging from the asymptomatic incidental lesion to symptomatic aortic rupture and cardiovascular collapse. The site of the lesion should also be taken into consideration, as PAU affecting the ascending aorta and arch are believed to be more at risk of complications, including rupture [3, 5], and thus surgical intervention is advocated even if the lesion is asymptomatic [7]. In summary, we have presented an unusual case of PAU affecting the aortic arch and a major branch of the aorta successfully managed with resection of all the lesions and interposition grafting.
References
Fig 3. Intraoperative photograph demonstrating two penetrating ulcers (*). The left affects the proximal brachiocephalic trunk, and the right affects the zenith of the aortic arch.
1. Stanson AW, Kazamier FJ, Hollier LH, et al. Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinical copathologic correlations. Ann Vasc Surg 1986;1: 15–23. 2. Vilacosta I, San Roman JA. Acute aortic syndrome. Heart 2001;85:365– 8. 3. Troxler M, Mavor AID, Homer-Vanniasinkam S. Br J Surg 2001;88:1169 –77. 4. Cho KR, Stanson AW, Potter DD, Cherry KJ, Schaff HV, Sundt TM 3rd. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch. J Thorac Cardiovasc Surg 2004;127: 1393– 401. 5. Sundt TM. Intramural hematoma and penetrating atherosclerotic ulcer of the aorta. Ann Thorac Surg 2007;83:S835– 41. 6. Chu B, Yuan C, Takaya N, Shewchuck JR, Clowes AW, Hatsukami TS. Serial high-spatial-resolution, multi-sequence magnetic resonance imaging studies identify fibrous cap rupture and penetrating ulcer into carotid atherosclerotic plaque. Circulation 2006;113:e660 –1. 7. Movsowitz HD, Lampert C, Jacobs LE, Kotler MN. Penetrating atherosclerotic aortic ulcers. Am Heart J 1994;128:1210 –7.
FEATURE ARTICLES
acute aortic syndromes [2]. The PAUs are typically seen in elderly patients with hypertension and atherosclerosis [3, 4]. Histologically they are described as an atherosclerotic lesion with ulceration that penetrates the internal elastic lamina and allows hematoma formation within the medial layer of the aortic wall [1]. It is well documented that a PAU can result in an intramural hematoma; however this is not universal, presumably as medial fibrosis secondary to chronic atherosclerosis prevents propagation of the hematoma [5]. Despite the evidence thus far, penetrating ulcers are still poorly characterized. It is unclear if they are related to atherosclerosis or other pathologic mechanisms. In our case, the presence of giant cells suggests an inflammatory component to the medial necrosis. This is further underlined by the fact that the lesions were found to be completely separate to the areas of atherosclerosis. However, the autoimmune profile and syphilis serology were both negative, which may suggest an atherosclerotic process. These findings further accentuate the fact that the cause of the penetrating ulcers is unclear, and whether the inflammatory component is the cause of the penetrating ulcers or secondary to the presence of penetrating ulcers further complicates an already poorly understood phenomenon. The PAUs are frequently observed in the descending thoracic and abdominal aorta; the ulcers are often multiple in number and range in size from 2 to 25 mm diameter and 4 to 30 mm in depth [3]. It is uncommon to find PAUs in the aortic arch. Troxler and colleagues [3] report 18 PAU within the arch from 130 patients affected. A similar review from the Mayo Clinic found PAU affecting the arch in only 9 of 119 patients [4]. The presence of multiple PAU within the arch in our patient could be described as unusual, but the occurrence of an ulcer involving the proximal brachiocephalic trunk makes this case almost unique. An exten-
CASE REPORT HICKEY ET AL MULTIPLE PENETRATING AORTIC ULCERS