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Fatal eosinophilic coronary periarteritis leads to dissection in a young patient: A case report Payman Izadpanah (MD)a, Sonia Shaabani (MD)a,*, Alireza Heiran (MD Candidate)b a b
Alzahra Cardiovascular Charitable Hospital, Shiraz University of Medical Sciences, Shiraz, Iran Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran
A R T I C L E I N F O
A B S T R A C T
Article history: Received 3 July 2019 Received in revised form 9 September 2019 Accepted 21 September 2019
Eosinophilic coronary periarteritis (ECPA) is a poorly studied type of coronary arteritis. It causes myocardial ischemia and most cases are diagnosed at autopsy. We report the case of a 35-year-old woman who presented with sudden unexpected cardiac arrest and was brought to the emergency ward. Cardiopulmonary resuscitation was started by ambulance paramedic. On arrival, the patient was transferred to the catheterization laboratory due to sudden aborted cardiac death. The angiography was performed and dissection of the left main coronary artery, extended to the left anterior descending artery was detected. Pathological study confirmed ECPA. It appears that young and healthy patients with a history of intermittent vasospastic angina should be evaluated for ECPA.
© 2019 Published by Elsevier Ltd on behalf of Japanese College of Cardiology.
Keywords: Eosinophilic coronary periarteritis Sudden unexpected death Spontaneous coronary artery dissection Vasospastic angina
Introduction
Case report
Eosinophilic coronary periarteritis (ECPA) is a rare cause of coronary arteritis, which is fatal. It causes ventricular arrhythmia, myocardial ischemia, and unexpected cardiac arrest in young and healthy patients with a history of intermittent vasospastic angina; hence, most cases are diagnosed at autopsy. It is frequently accompanied by spontaneous coronary artery dissection (SCAD), without any sign of vasculitis in other organs. In epidemiological aspect, patients with SCAD are mostly women (male/female ratio = 1:14) while isolated ECPA presents in men (male/female ratio = 8:3), predominantly [1]. We report the case of a 35-year-old woman who was brought to the emergency ward presenting with sudden unexpected cardiac arrest.
A 35-year-old depressed divorced woman who lived with her family, was brought by Emergency Medical Service (EMS) with aborted cardiac arrest. The chest pain started abruptly at rest in the afternoon that was followed by cardiac arrest at home, and immediately, a family member called EMS. The ambulance paramedic started cardiopulmonary cerebral resuscitation (CPCR). She did not have any history of asthma, skin rash, diabetes, hypertension, smoking, trauma to chest wall, or family history of premature coronary artery disease (CAD), as well as taking oral medicine or using nasal spray. Additionally, her family claimed that she had the episodic chest pain at rest especially during anger and depressed mood, which exacerbated within 2 weeks before the cardiac event. In physical examination in emergency ward of Alzahra Cardiovascular Charitable Hospital, affiliated to Shiraz University of Medical Sciences, Iran, weak peripheral pulse was detected, lung sound was clear, and jugular venous pressure (JVP) was normal. The electrocardiogram (ECG) showed bradycardia and non-specific findings. Echocardiography showed severely decreased left ventricular systolic function. There was no evidence of aortic root dilation or flap in ascending aorta, pericardial
* Corresponding author at: Alzahra Cardiovascular Charitable Hospital, Shiraz University of Medical Sciences, Shiraz, 7164954937, Iran. E-mail address: [email protected] (S. Shaabani).
https://doi.org/10.1016/j.jccase.2019.09.014 1878-5409/© 2019 Published by Elsevier Ltd on behalf of Japanese College of Cardiology.
Please cite this article in press as: Izadpanah P, et al. Fatal eosinophilic coronary periarteritis leads to dissection in a young patient: A case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.09.014
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Fig. 1.
Angiography findings: (A) coronary spasm vs. dissection of left coronary artery (LCA) extended to left anterior descending artery (LAD) before nitroglycerin administration; (B) narrowing of LCA after nitroglycerin administration, no difference to photo (A) since pathology was dissection not only coronary spasm; (C) attempt to stent LCA and LAD during cardiopulmonary cerebral resuscitation; (D) passing the wire from LCA to LAD during the procedure followed by the stenting failure; (E) no visible flap in ascending aorta that type A dissection was ruled out [Copyright: © Izadpanah et al.].
Please cite this article in press as: Izadpanah P, et al. Fatal eosinophilic coronary periarteritis leads to dissection in a young patient: A case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.09.014
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effusion, and visible clot in left ventricle, other chambers and pulmonary arteries. Due to hemodynamic instability, CPCR was started, inotrope was administered, and the patient was transferred to the catheterization laboratory for emergency angioplasty. In the first injection, a radiolucent linear defect suggestive of an intimal dissection involving left main coronary artery (LCA) extended to left anterior descending artery (LAD) was detected. To eliminate coronary spasm, intracoronary nitroglycerin was injected, but no difference was evident; however, the possible effect of intracoronary nitroglycerin cannot be judged since pathology was dissection not only coronary spasm. Then the coronary arteries were wired from LCA to LAD. But the interventionist could not do stenting or ballooning of the coronary vessel, due to the storm ventricular arrhythmia and unsuccessful CPCR (Fig. 1). Finally, the patient’s condition worsened and regardless of performing CPCR, she expired. The weight of the heart was 320 g on autopsy. In addition, her complete blood count did not show eosinophilia, and only microcytic anemia was read. Pathology study confirmed ECPA. The gross pathology was the development of a dissection plane between media and adventitia of the affected coronary arteries with a compression effect on the true lumen by hematoma in the false lumen. Microscopic examination of LAD segment showed the pathologic hallmark of ECPA; that is a dense circumferentially inflammatory infiltration, mainly composed of eosinophils which is seen in the adventitia and periadventitial fibroadipose tissue of the dissected coronary artery (Fig. 2).
Fig. 2.
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Discussion Although atherosclerotic plaque rupture is the main cause of myocardial infarction in geriatrics, among the young and healthy patients coronary artery spasm, coronary artery vasculitis, cocaine abuse, and external chest compression should be considered [2]. ECPA is a vasculitis and is one of the causes of unexpected cardiac arrest amongst young and healthy patients [3]. ECPA is probably a distinct type of coronary arteritis and it should be differentiated from the other types of vasculitis, such as isolated (limited) or medium-sized arteritis like polyarteritis nodosa (PAN), granulomatosis with polyangiitis (Wegener's granulomatosis), allergic granulomatous angiitis (or Churg–Strauss Syndrome) [1], and Kounis syndrome [4]. In other words, a clinician should consider the non-atherosclerotic coronary spasm etiologies in a young patient, and meticulously differentiate them as well. The above-mentioned vasculitides involve small- and mediumsize vessels. However, in some points these are different from ECPA: first, the arterial walls exhibit fibrinoid necrosis associated with coronary thrombosis; and second, these systemic vasculitides can involve lung, paranasal sinus, and kidney (glomerulonephritis), with a positive serology marker (PANCA-CANCA) and eosinophilia greater than 10%. However, PAN can be manifested as an isolated form, involving coronary arteries with luminal thrombosis without other systemic manifestations. Also, giant cell vasculitis such as Takayaso vasculitis can involve the ostium of coronary arteries [5,6].
Pathology findings: Microscopic examination of left anterior descending artery segment with gross abnormalities shows dense circumferentially inflammatory infiltration, mainly composed of eosinophils which is seen in the adventitia and periadventitial fibroadipose tissue [Copyright: © Izadpanah et al.].
Please cite this article in press as: Izadpanah P, et al. Fatal eosinophilic coronary periarteritis leads to dissection in a young patient: A case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.09.014
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The other differential diagnosis for such unexpected sudden death in a young patient is Kounis syndrome. It is noticed that a quarter of the patients have a positive history of allergic manifestations. It is postulated that the coronary spasm originates from a hypersensitivity reaction to drugs, especially antibiotics, and insect bites. And in approximately 80% of cases, it occurs with the first exposure. Worth noting, the patient experiences skin rash, wheezing, hives, chest pain, and palpitation [4]. In a patient with ECPA, the inflammatory infiltration is predominately composed of eosinophils in the adventitia and periadventitia in the absence of fibrinoid necrosis or granuloma [7]. Also, mast cell densities are approximately three fold higher in dissected portion, which induce degranulation, eosinophilmediated inflammatory process, coronary spasm, chest pain, and arrhythmia [8]. However, the pathophysiology of ECPA remains a puzzle. ECPA is characterized by an evening-to-early morning intermittent vasospastic angina, the so called Prinzmetal’s vasospastic angina. By and large, when a young patient without traditional risk factors for CAD, and occasionally with a positive history of asthma or allergy, presents with a vasospastic angina, epicardial coronary arteries’ inflammation due to ECPA should be assessed. Since ECPA is an unexpected and life-threatening condition, developing a diagnostic tool amongst suspicious young and healthy patients is demanded. Positron emission-computed tomography (PET/CT) is a novel modality in cardiology that is superior to other tools in evaluating cardiac perfusion in combination with assessment of epicardial coronary arteries’ anatomy and inflammation of vessel wall in patients who are suspected of having CAD [9]. It can yield information about the infiltrative cells in the vessels and myocardium in early stages of cardiac inflammatory disease, as it has a diagnostic role in other vasculitis, for instance Wegener's granulomatosis or Churg– Strauss Syndrome and cardiac sarcoidosis. Thereby, it might be an interesting choice [10]. However, the accuracy of PET/CT on small vessels such as coronary arteries is questionable and further studies are warranted.
Conclusion ECPA should be of sufficient concern and managed amongst young and healthy patients who have a positive history of intermittent Prinzmetal vasospastic angina and lack traditional risk factors for CAD. It is confirmed by autopsy to date, hence developing a tool in detecting this poorly studied fatal coronary arteritis is demanded. PET/CT might be an interesting tool, since it can detect infiltrative cells preceding sudden cardiac death. Conflict of interest The authors declare no conflict of interest. References [1] Kajihara H, Tachiyama Y, Hirose T, Takata A, Saito K, Murai T, et al. Eosinophilic coronary periarteritis (vasospastic angina and sudden death), a new type of coronary arteritis: report of seven autopsy cases and a review of the literature. Virchows Arch 2013;462:239–48. [2] Kajihara H, Kato Y, Takanashi A, Nakagawa H, Tahara E, Otsuki T, et al. Periarteritis of coronary arteries with severe eosinophilic infiltration: a new pathologic entity (eosinophilic periarteritis)? Pathol Res Pract 1988;184:46–52. [3] Omalu B, Hammers J, DiAngelo C, Moore S, Luckasevic T. Autopsy features of sudden death due to isolated eosinophilic coronary arteritis: report of two cases. J Forensic Nurs 2011;7:153–6. [4] Abdelghany M, Subedi R, Shah S, Kozman H. Kounis syndrome: a review article on epidemiology, diagnostic findings, management and complications of allergic acute coronary syndrome. Int J Cardiol 2017;232:1–4. [5] Triantafyllis AS, Sakadakis EA, Papafilippaki A, Katsimbri P, Panou F, Anastasiou-Nana M, et al. Churg–Strauss syndrome masquerading as an acute coronary syndrome. Am J Emerg Med 2015;33(313):e5–6. [6] Chai JT, McGrath S, Lopez B, Dworakowski R. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report. Eur Heart J Case Rep 2018;2. yty075. [7] Carreon CK, Esposito MJ. Eosinophilic coronary monoarteritis. Arch Pathol Lab Med 2014;138:979–81. [8] Mandal R, Brooks EG, Corliss RF. Eosinophilic coronary periarteritis with arterial dissection: the mast cell hypothesis. J Forensic Sci 2015;60:1088–92. [9] Di Carli MF, Murthy VL. Cardiac PET/CT for the evaluation of known or suspected coronary artery disease. Radiographics 2011;31:1239–54. [10] Miyagawa M, Yokoyama R, Nishiyama Y, Ogimoto A, Higaki J, Mochizuki T. Positron emission tomography-computed tomography for imaging of inflammatory cardiovascular diseases. Circ J 2014;78:1302–10.
Please cite this article in press as: Izadpanah P, et al. Fatal eosinophilic coronary periarteritis leads to dissection in a young patient: A case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.09.014