Case Report

Kounis syndrome captured by coronary angiography computed tomography

Abstract

Kounis syndrome is the concurrence of acute coronary syndrome with mast cell activation induced by inflamma- tory mediators released during an allergic reaction. Two types of Kounis syndrome have been described. In type II, Coronary spasm occurs adjacent to a preexisting atheroma- tous lesion in which allergic stimulation results in plaque erosion or rupture leading to myocardial infarction (MI). Herein, we report a case of contrast-induced Kounis syndrome type II and provide Coronary angiography computed tomography images of the vaso- spasm as the Hypersensitivity reaction evolved during the imaging procedure.

A 54-year-old man presented with flushing, shortness of breath, and severe Retrosternal chest pain radiating to his neck 30 minutes after receiving contrast media for CAG CT. An electrocardiogram revealed ST elevation in leads V2 to V5 with reciprocal changes in II, III, and aVF, compatible with anterior acute MI. Coronary angiography performed 1 hour after the onset of symptoms revealed subtotal occlusion of the proximal left anterior descending artery, and a LAD stent was subsequently placed. Coronary angiography CT performed immediately after the onset of the hypersensitivity reaction demonstrated diffuse spasm of the proximal LAD without occlusion.

After a thorough literature review, we were unable to identify any cases wherein an image of the coronary artery was captured immediately after the onset of the allergic reaction. In this case, we could confirm the course of Kounis syndrome type II from the Coronary vasospasm to the rupture of an atheroma through serial imaging studies.

Kounis syndrome is the concurrence of acute coronary syndrome with mast cell activation induced by inflam- matory mediators released during an allergic reaction. First described in 1991 by Kounis, it has also been termed “Allergic angina” [1,2]. To date, there have been several reported cases of Kounis syndrome after exposure to several drugs, ingestion of shellfish, and stings from vipers, bees, hymenoptera, and jellyfish [3-8]. However,

after a thorough literature review, we were unable to identify any cases wherein an image of the coronary artery was captured immediately after the onset of the allergic reaction. Herein, we report a case of contrast- induced Kounis syndrome and provide CAG CT images of the vasospasm as the hypersensitivity reaction evolved during the imaging procedure.

A 54-year-old man with a medical history significant for medication-controlled diabetes mellitus and minimal coronary artery obstructive disease diagnosed by CAG 10 years previously was transferred from the Department of Radiology to the emergency department (ED) due to sudden onset of chest pain. Though his coronary artery obstructive disease had remained asymptomatic, he elected to undergo CAG CT for a routine medical follow-up. Immediately after injection of the CT contrast media, he complained of flushing, shortness of breath, and chest discomfort, which became increasingly severe during the course of 10 minutes. Therefore, he was transferred to the ED for further evaluation.

On presentation to the ED, his blood pressure was 40/30 mm Hg with a heart rate of 100 beats/min. He complained of severe retrosternal chest pain radiating to the neck, accompanied by dyspnea, diaphoresis, facial swelling, and a pruritic skin rash. An electrocardiogram (Fig. 1) revealed ST-segment elevation in leads V2 to V5 with Reciprocal changes in II, III, and aVF, compatible with an anterior acute MI. He was given rapid hydration with normal saline, and some medications were administered intravenously: 125 mg methylprednisolone sodium succinate, 4 mg chlorpenir- amine maleate, 200 mg cimetidine, 3 mg morphine, and a bolus of 5000 unit heparin. He was also given 100 mg of aspirin and 600 mg of clopidogrel. A venous blood sample was obtained, after which he was transferred to the coronary care unit where CAG was attempted within 1 hour after the onset of pain. The CAG demonstrated a subtotal occlusion of the proximal LAD artery. Subsequently, percutaneous trans- luminal coronary angioplasty (PTCA) with stent insertion into the LAD was successfully performed (Fig. 2). The results of the initial laboratory studies revealed normal cardiac enzymes, including troponin T. Eight hours later, the troponin T level was noted to be elevated to 0.04 mc/L. After PTCA, the patient’s condition improved with ST-elevation resolution (Fig. 3). Transthoracic echocardiography obtained the

0735-6757/$ – see front matter (C) 2010

Fig. 1 Electrocardiogram showing ST-segment elevation in leads V2 to V5 (arrow) with reciprocal changes in II, III, and aVF (arrow head), compatible with an anterior acute MI.

following day revealed no regional Wall motion abnormality and an ejection fraction of 75%. The patient was discharged on hospital day 4 without further incident.

Fig. 2 Coronary angiography revealing subtotal occlusion of the proximal LAD artery (arrow).

The result of the CAG CT, performed immediately before presentation to the ED, revealed diffuse spasm of the proximal LAD (Fig. 4).

The trigger for this hypersensitivity reaction was thought to be mast cells located between cardiomyocytes in the internal layer of arteries and around coronary vessels. In the course of an Anaphylactic reaction, Mast cell degranulation results in the release of several vasoactive and collagen- degrading compounds, which leads to a powerful vasocon- striction of the coronary arteries [9]. Although mast cells have been found in the hearts of healthy humans, a greater number are located in the intima and adventitia of vessels with preexisting atheromatic lesions, particularly in marginal regions of atherosclerotic plaques [10]. Proteolytic enzymes, such as chymase and tryptase, released from stimulated mast cells degrade connective tissue covering the atheromatous plaque, making it vulnerable to rupture [9,11]. Based on this preexistent dichotomy, 2 types of Kounis syndrome have been described. Type I occurs in patients with Normal coronary arteries in whom the acute release of Inflammatory mediators induces coronary artery spasm. In type II, coronary spasm occurs adjacent to a preexisting atheroma- tous lesion in which allergic stimulation results in plaque erosion or rupture leading to MI [1,12].

The case herein was an example of type II Kounis syndrome. The ST-elevation MI developed after the administration of the contrast media that provoked an anaphylactoid reaction including coronary mast cell

Fig. 3 Electrocardiogram showing complete ST-segment resolution after PTCA.

activation, leading to coronary artery spasm detectable on CAG CT. Initially, the blood flow within the LAD was preserved; however, over time, the atheromatous plaque became vulnerable to rupture, ultimately leading to near- total occlusion of the vessel. Coronary angiography performed 1 hour after the onset of symptoms confirmed the presence of subtotal proximal LAD occlusion. Through

Fig. 4 Coronary angiography CT revealing diffuse spasm of the proximal LAD (arrow head) before the presentation to the ED.

serial imaging studies, we could confirm the course of Kounis syndrome type II from the coronary vasospasm to the rupture of an atheroma.

Joon Min Park MD Junho Cho MD Sung Pil Chung MD

College of Medicine Yonsei University

Seoul, South Korea

Min Joung Kim MD

College of Medicine Yonsei University

Seoul, South Korea Department of Emergency Medicine Eonjuro 712, Gangnam-gu

135-720, Seoul, South Korea E-mail address: [email protected]

doi:10.1016/j.ajem.2009.08.018

References

1. Sinkiewicz W, Sobanski P, Bartuzi Z. Allergic myocardial infarction. Cardiol J 2008;15:220-5.
2. Kounis NG, Zavras GM. Histamine-induced coronary artery spasm: the concept of allergic angina. Br J Clin Pract 1991;45:121-8.
3. Frangides C, Kouni S, Niarchos C, et al. Hypersersensitivity and Kounis syndrome due to a viper bite. Eur J Intern Med 2006;17: 215-6.
4. Ioannidis TI, Mazarakis A, Notaras SP, et al. Hymenoptera sting- induced Kounis syndrome: effects of aspirin and beta-blocker administration. Int J Cardiol 2007;121:105-8.
5. Kogias JS, Sideris SK, Anifadis SK. Kounis syndrome associated with hypersensitivity to hymenoptera stings. Int J Cardiol 2007;114:252-5.
6. Zavras GM, Papadaki PJ, Kokkinis CE, et al. Kounis syndrome secondary to allergic reaction following shellfish ingestion. Int J Clin Pract 2003;57:622-4.
7. Tavil Y, Turfan M, Turkoglu S, et al. Kounis syndrome

secondary to amoxicillin/clavulanic acid use. Int J Cardiol 2008; 124:4-7.

1. Mytas DZ, Stougiannos PN, Zairis MN, et al. Acute anterior myocardial infarction after multiple Bee stings. A case of Kounis syndrome. Int J Cardiol 2008.
2. Laine P, Kaartinen M, Penttila A, et al. Association between myocardial infarction and the mast cells in the adventitia of the infarct-related coronary artery. Circulation 1999;99:361-9.
3. Atkinson JB, Harlan CW, Harlan GC, et al. The association of mast cells and atherosclerosis: a morphologic study of early atherosclerotic lesions in Young people. Human Pathology 1994;25:154-9.
4. Kovanen PT, Kaartinen M, Paavonen T. Infiltrates of activated mast cells at the site of coronary atheromatous erosion or rupture in myocardial infarction. Circulation 1995;92:1084-8.
5. Nikolaidis LA, Kounis NG, Gradman AH. Allergic angina and allergic myocardial infarction: a new twist on an old syndrome. Can J Cardiol 2002;18:508-11.