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Case Study
Sarcoidosis or acute coronary syndrome: Easily distinguishable?
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314538036 aan.sagepub.com
William Dominic Marley, Karen Booth and Reuben Jeganathan
Abstract Sinus of Valsalva aneurysm is a rare presenting feature of ST-elevation myocardial infarction. We describe such a case which resolved with thrombolysis, and on further investigation, the patient was found to have a large sinus of Valsalva aneurysm. Abnormal gross pathology at the time of surgery was suspected to be aortitis which was only confirmed on postmortem examination 6 months later. We highlight the importance of working closely with a pathologist when aortitis is suspected, because failure to treat it medically results in a poor outcome.
Keywords Aortic aneurysm, Aortitis, Myocardial infarction, Sarcoidosis, Sinus of Valsalva, Vasculitis
Case report A 47-year-old gentleman of Asian descent presented with acute inferior ST-elevation myocardial infarction with raised troponin levels. His past medical history included sleep apnea, type 2 diabetes, gout, and rheumatic fever. Following treatment, his condition improved. Surprisingly, angiography showed no coronary disease. Computed tomography of the chest and an echocardiogram revealed a sinus of Valsalva aneurysm and hilar lymphadenopathy (Figure 1). There was severe aortic incompetence with an end-diastolic dimension of 7.1 cm and end-systolic dimension of 5.8 cm, with evidence of moderate impairment of left ventricular function (ejection fraction 45%). Clinical examination did not reveal the typical stigmata of connective tissue disease, tuberculosis, sarcoidosis, or heart failure. At sternotomy, a large sinus of Valsalva aneurysm involving the right coronary cusp was revealed. Standard cardiopulmonary bypass was instituted with both antegrade and retrograde cardioplegia and core cooling to 32 C. Macroscopically, the aorta was heavily diseased with tissue thickness >5 mm involving the coronary ostia (Figure 2). An uncomplicated modified Bentall procedure was performed and the patient came off bypass at the first attempt on minimal inotropic support (noradrenalin 0.04 mg kg 1 mL 1, dopamine 6 mg kg 1 mL 1). He was discharged home on the 6th postoperative day. Histopathology showed
degeneration of the aortic wall with fibrosis and myxoid degeneration. There was no evidence of primary aortitis in the samples sent for pathology. The patient was readmitted with a cerebrovascular accident 6 months postoperatively. An echocardiogram revealed a false aneurysm arising from the aortic root. This was confirmed on subsequent contrast computed tomography (Figure 3). He died shortly after this. A postmortem examination confirmed the hilar lymphadenopathy to be sarcoidosis with severe aortitis involving both coronary ostia, resulting in a large false aneurysm causing compression of the pulmonary artery with cardiac ischemia and a final fatal myocardial infarction. No cerebrovascular accident was confirmed at autopsy.
Discussion The majority of sinus of Valsalva aneurysms arise from the noncoronary sinus of Valsalva (65%– 85%).1Predominantly found in males and in the
Department of Cardiothoracic Surgery, Royal Victoria Hospital, Belfast, UK Corresponding author: William Dominic Marley, Department of Cardiothoracic Surgery, Royal Victoria Hospital, 274 Grosvenor Road, Belfast BT12 6BA, UK. Email: [email protected]
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[1–3] [PREPRINTER stage]
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Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Preoperative computed tomography demonstrating an enlarged aortic root.
Figure 2. Operative findings of grossly inflamed tissue that could not be totally excised.
Figure 3. Contrast computed tomography on readmission, showing a hematoma surrounding the aortic root, with leakage arising from the native coronary buttons.
Asian population, cases of unruptured sinus of Valsalva aneurysm presenting with a non-ST-elevation myocardial infarction have been described previously, but those resulting in ST-elevation myocardial
infarction are very rare.2 Congenital and acquired sinus of Valsalva aneurysms are considered to have differing pathological features, with congenital aneurysms being associated with weakness of the aortic media, and acquired aneurysms occurring as the result of damage to the vessel wall. This may be secondary to infection, trauma, atherosclerosis, or connective tissue disorders Sarcoidosis-linked aortitis is uncommon, according to a case series and literature review in 2000.3 Sarcoidosis is known to mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, and Takayasu’s arteritis, and it has been found to be disproportionately represented in African-American and Asian populations. The underlying disease process in this case was localized to the root of the aorta and native coronary arteries, and was not displayed elsewhere, with no other vasculitis seen at postmortem examination. In this case, the diagnosis of sarcoidosis was well-established by the typical histopathological findings, including necrotizing sarcoid granulomatosis in the native coronary tissue. Involvement or a relationship with vasculitis has been seen in documented cases.3 Its presence in the preoperative period and the gross pathology displayed in the operating room should forewarn of the risk of aortitis. On reflection, a history of fever and night sweats as signs of generalized inflammation were present in this patient’s history. Hilar lymphadenopathy and vasculitis may be coincidental or may have an immunological relationship, but no clear answer can be given on this.4 The case presented here and the few additional patients reported with sarcoidosis preceding vasculitis, may have had related diseases. Reports on Crohn’s disease, another granulomatous disorder with the development of arteritis, further supports the possibility of a common basis of granulomatous disorders and arteritis.5 Treatment with corticosteroids for vasculitis are described as palliative at best, with permanent remission uncommon. In selected cases with large vessel aneurysms, surgical therapy appears to be life-saving, but as this case illustrates, without histopathological confirmation, the surgical team are left with only a clinical suspicion. Sinus of Valsalva aneurysm caused by sarcoidosis vasculitis is rare, but it must be remembered that this granulomatous disease of unknown etiology may affect most organs including the heart and large or small blood vessels. Concomitant medical therapy in conjunction with surgical excision, if appropriate, is needed for a successful outcome. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
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(AAN)
[1–3] [PREPRINTER stage]
Marley et al.
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Conflict of interest statement None declared.
References 1. Meier JH, Seward JB, Miller FA, Oh JK and EnriquezSarano M. Aneurysms in the left ventricular outflow tract: clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr 1998; 11: 729–745. 2. Honda K, Nakamura Y, Tagusari O and Nakano K. Large sinus of Valsalva aneurysm causing myocardial infarction. Asian Cardiovasc Thorac Ann 2012; 20: 66–67.
3. Fernandes SR, Singsen BH and Hoffman GS. Sarcoidosis and systemic vasculitis. Semin Arthritis Rheum 2000; 30: 33–46. 4. Weiler V, Redtenbacher S, Bancher C, Fischer MB and Smolen JS. Concurrence of sarcoidosis and aortitis: case report and review of the literature. Ann Rheum Dis 2000; 59: 850–853. 5. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med 1994; 120: 919–929.
Downloaded from aan.sagepub.com at DEAKIN UNIV LIBRARY on November 15, 2015
(AAN)
[1–3] [PREPRINTER stage]
Case Study
Sarcoidosis or acute coronary syndrome: Easily distinguishable?
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314538036 aan.sagepub.com
William Dominic Marley, Karen Booth and Reuben Jeganathan
Abstract Sinus of Valsalva aneurysm is a rare presenting feature of ST-elevation myocardial infarction. We describe such a case which resolved with thrombolysis, and on further investigation, the patient was found to have a large sinus of Valsalva aneurysm. Abnormal gross pathology at the time of surgery was suspected to be aortitis which was only confirmed on postmortem examination 6 months later. We highlight the importance of working closely with a pathologist when aortitis is suspected, because failure to treat it medically results in a poor outcome.
Keywords Aortic aneurysm, Aortitis, Myocardial infarction, Sarcoidosis, Sinus of Valsalva, Vasculitis
Case report A 47-year-old gentleman of Asian descent presented with acute inferior ST-elevation myocardial infarction with raised troponin levels. His past medical history included sleep apnea, type 2 diabetes, gout, and rheumatic fever. Following treatment, his condition improved. Surprisingly, angiography showed no coronary disease. Computed tomography of the chest and an echocardiogram revealed a sinus of Valsalva aneurysm and hilar lymphadenopathy (Figure 1). There was severe aortic incompetence with an end-diastolic dimension of 7.1 cm and end-systolic dimension of 5.8 cm, with evidence of moderate impairment of left ventricular function (ejection fraction 45%). Clinical examination did not reveal the typical stigmata of connective tissue disease, tuberculosis, sarcoidosis, or heart failure. At sternotomy, a large sinus of Valsalva aneurysm involving the right coronary cusp was revealed. Standard cardiopulmonary bypass was instituted with both antegrade and retrograde cardioplegia and core cooling to 32 C. Macroscopically, the aorta was heavily diseased with tissue thickness >5 mm involving the coronary ostia (Figure 2). An uncomplicated modified Bentall procedure was performed and the patient came off bypass at the first attempt on minimal inotropic support (noradrenalin 0.04 mg kg 1 mL 1, dopamine 6 mg kg 1 mL 1). He was discharged home on the 6th postoperative day. Histopathology showed
degeneration of the aortic wall with fibrosis and myxoid degeneration. There was no evidence of primary aortitis in the samples sent for pathology. The patient was readmitted with a cerebrovascular accident 6 months postoperatively. An echocardiogram revealed a false aneurysm arising from the aortic root. This was confirmed on subsequent contrast computed tomography (Figure 3). He died shortly after this. A postmortem examination confirmed the hilar lymphadenopathy to be sarcoidosis with severe aortitis involving both coronary ostia, resulting in a large false aneurysm causing compression of the pulmonary artery with cardiac ischemia and a final fatal myocardial infarction. No cerebrovascular accident was confirmed at autopsy.
Discussion The majority of sinus of Valsalva aneurysms arise from the noncoronary sinus of Valsalva (65%– 85%).1Predominantly found in males and in the
Department of Cardiothoracic Surgery, Royal Victoria Hospital, Belfast, UK Corresponding author: William Dominic Marley, Department of Cardiothoracic Surgery, Royal Victoria Hospital, 274 Grosvenor Road, Belfast BT12 6BA, UK. Email: [email protected]
Downloaded from aan.sagepub.com at DEAKIN UNIV LIBRARY on November 15, 2015
XML Template (2014) [28.5.2014–12:10pm] //blrnas3/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/140118/APPFile/SG-AANJ140118.3d
(AAN)
[1–3] [PREPRINTER stage]
2
Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Preoperative computed tomography demonstrating an enlarged aortic root.
Figure 2. Operative findings of grossly inflamed tissue that could not be totally excised.
Figure 3. Contrast computed tomography on readmission, showing a hematoma surrounding the aortic root, with leakage arising from the native coronary buttons.
Asian population, cases of unruptured sinus of Valsalva aneurysm presenting with a non-ST-elevation myocardial infarction have been described previously, but those resulting in ST-elevation myocardial
infarction are very rare.2 Congenital and acquired sinus of Valsalva aneurysms are considered to have differing pathological features, with congenital aneurysms being associated with weakness of the aortic media, and acquired aneurysms occurring as the result of damage to the vessel wall. This may be secondary to infection, trauma, atherosclerosis, or connective tissue disorders Sarcoidosis-linked aortitis is uncommon, according to a case series and literature review in 2000.3 Sarcoidosis is known to mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, and Takayasu’s arteritis, and it has been found to be disproportionately represented in African-American and Asian populations. The underlying disease process in this case was localized to the root of the aorta and native coronary arteries, and was not displayed elsewhere, with no other vasculitis seen at postmortem examination. In this case, the diagnosis of sarcoidosis was well-established by the typical histopathological findings, including necrotizing sarcoid granulomatosis in the native coronary tissue. Involvement or a relationship with vasculitis has been seen in documented cases.3 Its presence in the preoperative period and the gross pathology displayed in the operating room should forewarn of the risk of aortitis. On reflection, a history of fever and night sweats as signs of generalized inflammation were present in this patient’s history. Hilar lymphadenopathy and vasculitis may be coincidental or may have an immunological relationship, but no clear answer can be given on this.4 The case presented here and the few additional patients reported with sarcoidosis preceding vasculitis, may have had related diseases. Reports on Crohn’s disease, another granulomatous disorder with the development of arteritis, further supports the possibility of a common basis of granulomatous disorders and arteritis.5 Treatment with corticosteroids for vasculitis are described as palliative at best, with permanent remission uncommon. In selected cases with large vessel aneurysms, surgical therapy appears to be life-saving, but as this case illustrates, without histopathological confirmation, the surgical team are left with only a clinical suspicion. Sinus of Valsalva aneurysm caused by sarcoidosis vasculitis is rare, but it must be remembered that this granulomatous disease of unknown etiology may affect most organs including the heart and large or small blood vessels. Concomitant medical therapy in conjunction with surgical excision, if appropriate, is needed for a successful outcome. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Downloaded from aan.sagepub.com at DEAKIN UNIV LIBRARY on November 15, 2015
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(AAN)
[1–3] [PREPRINTER stage]
Marley et al.
3
Conflict of interest statement None declared.
References 1. Meier JH, Seward JB, Miller FA, Oh JK and EnriquezSarano M. Aneurysms in the left ventricular outflow tract: clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr 1998; 11: 729–745. 2. Honda K, Nakamura Y, Tagusari O and Nakano K. Large sinus of Valsalva aneurysm causing myocardial infarction. Asian Cardiovasc Thorac Ann 2012; 20: 66–67.
3. Fernandes SR, Singsen BH and Hoffman GS. Sarcoidosis and systemic vasculitis. Semin Arthritis Rheum 2000; 30: 33–46. 4. Weiler V, Redtenbacher S, Bancher C, Fischer MB and Smolen JS. Concurrence of sarcoidosis and aortitis: case report and review of the literature. Ann Rheum Dis 2000; 59: 850–853. 5. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med 1994; 120: 919–929.
Downloaded from aan.sagepub.com at DEAKIN UNIV LIBRARY on November 15, 2015
