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Kounis syndrome: a forgotten cause of chest pain/cardiac chest pain in children/Kounis sendromu: Gogus agrisinin unutulan bir sebebi/ cocuklarda kardiyak gogus agrisi.

Dear Editor,

I read with interest the article "Cardiac chest pain in children" by Cagdas et al. (1) which has retrospectively evaluated 120 children admitted to a pediatric cardiology clinic with chest pain. Although chest pain in children is rarely reported to be associated with cardiac diseases in the literature (2) authors have found that 52 of the patients (42.5%) had cardiac diseases and 28 (23.3%) of these patients' cardiac diseases were thought to directly cause their chest pain. These higher ratios may result experience of a tertiary referral centre. Although a variety of diseases is described as the causes of the chest pain in the present study, I want to remind a forgotten cause of chest pain in children.

Kounis syndrome (allergic angina or allergic myocardial infarction) is the coincidental occurrence of chest pain and allergic reactions accompanied by clinical and laboratory findings of classical angina pectoris caused by inflammatory mediators released during an allergic insult (3). There are several factors, which have been reported as capable of inducing Kounis syndrome. These include a number of conditions, several drugs, foods, insect stings and etc. The mechanism of Kounis syndrome is coronary artery spasm due to mast cells degranulation and the subsequent release of vasoactive mediators (3).

Three variants of Kounis syndrome have been described (3-5). Type I, occurring in patients with angiographically normal coronary arteries, and type II, occurring in patients in whom concomitant atheromatous lesions are found. Type III variant includes patients with coronary artery stent thrombosis in whom aspirated thrombus specimens stained with hematoxylin-eosin and Giemsa demonstrate the presence of eosinophils and mast cells. However, since type II and type III variants include patients with preexisting atheromatous disease, I think that it is almost impossible to encounter the type II and type III variants in childhood.

After the first report of Kounis syndrome in a 13-year-old boy (6), we have recently published first children with Kounis syndrome in the literature (7). Although nearly 300 cases with Kounis syndrome have been reported in the literature I think that many of the cases are misdiagnosed or unreported.

I want to emphasize that pediatricians should be aware of the allergic myocardial infarction. The diagnosis of this unique disease should be entertained when acute-onset chest pain is accompanied by allergic symptoms, electrocardiographic changes and elevated cardiac enzymes. All adults and children admitted to the emergency departments with chest pain and ST elevation on electrocardiography, should be interrogated for allergic insults. Kounis syndrome is not a very rare disease but a 'very rarely diagnosed' disease.

Murat Biteker

Department of Cardiology, Kartal Kosuyolu Heart Education and Research Hospital, Istanbul, Turkey


(1.) Cagdas. DN, Pac FA. Cardiac chest pain in children. Anadolu Kardiyol Derg 2009;9:401-6.

(2.) Coleman WL. Recurrent chest pain in children. Pediatr Clin North Am 1984;31:1007-26.

(3.) Kounis NG: Kounis syndrome (allergic angina and allergic myocardial infarction): a natural paradigm? Int J Cardiol 2006; 7:7-14.

(4.) Biteker M, Duran NE, Biteker E Civan HA, Gunduz S, Grjkdeniz T, et al. Kounis syndrome: first series in Turkish patients. Anadolu Kardiyol Derg 2009;9:59-60.

(5.) Biteker M. A new classification of Kounis syndrome. Int J Cardiol 2010 Jun 7. [Epub ahead of print].

(6.) Biteker M, Duran NE, Erturk E, Aykan AC, Civan HA, et al. Kounis Syndrome secondary to amoxicillin/clavulanic acid use in a child. Int J Cardiol 2009;136:e3-5.

(7.) Biteker M, Eksj Duran N, Sungur Biteker E Ayyildiz Civan H, Kaya H, Gokdeniz T, et al. Allergic myocardial infarction in childhood: Kounis syndrome. Eur J Pediatr 2010; 169: 27-9.

Address for Correspondence / Yazisma Adresi: Dr. Murat Biteker Kartal Kosuyolu Heart Education and Research Hospital, Cardiology, Istanbul, Turkey

Phone: +90 216 488 80 02 Fax: +90 216 459 63 21



Author's Reply

Dear Editor,

We thank author of the letter for their valuable comments for our study. In our study, we found that the risks of structural cardiac disease and arrhythmia and the admission with chest pain increase with increasing age in children. In addition, we found a high ratio of cardiac disease in children with chest pain. First reason of this may be the high frequency of some cardiac diseases in children such as MVP or ventricular/supraventricular ectopies. Second, some of our patients were referred patients. Third and the most important reason is the standard evaluation of patients, using our algorithm for chest pain, with standard questions for evaluation and standard diagnostic tests.

In the study of Selbst et al. (1), the frequency of cardiac pathology was found to be 4%, but they reported in their study that fifty three percent of the patients were not evaluated by electrocardiography (ECG). So, the frequency of cardiac pathology in their study may not reflect the real frequency. In our study, all patients were evaluated with ECG. Kounis syndrome, which is the result of ischemic changes caused by inflammatory mediators released during allergic reactions, may be suspected only, if the patient is evaluated with ECG. In our study, our patients did not have both electrocardiographic findings of ischemia and allergic symptoms.

There are many different causes of chest pain in children like Kounis syndrome (2,3), so there is need for further investigations in the evaluation of chest pain in children. Using an algorithm with standard workup will make the evaluation easier in the outpatient clinics.

Deniz Cagdas Ayvaz

Section of Pediatric Cardiology, Turkiye Yiiksek ihtisas Education and Research Hospital, Ankara, Turkey


(1.) Selbst SM, Ruddy RM, Clark BJ, Henretlg FM, Santulli T Jr. Pediatric chest pain: a prospective study. Pediatrics 1988; 82:319-23.

(2.) Biteker M, Eksj Duran N, Sungur Biteker E Erturk E, Aykan AC, Civan HA, et al. Kounis Syndrome secondary to amoxicillin/clavulanic acid use in a child. Int J Cardiol 2009;136:e3-5.

(3.) Pac FA, Cagdas. DN, Ulas M, Ozatik MA, Pac M. Left main coronary artery and aortic root compression associated with atrial septal defect and pulmonary hypertension. Int J Cardiol 2007; 118:e 41-3.

Address for Correspondence / Yazisma Adresi: Dr. Deniz Cagdas Ayvaz

Section of Pediatric Cardiology, Turkiye Yuksek Ihtisas Education and Research Hospital, Ankara, Turkey

Phone: +90 312 306 17 24 Fax: +90 312 312 41 20

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Article Details
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Title Annotation:Letters to the Editor/Editore Mektuplar
Author:Biteker, Murat
Publication:The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)
Article Type:Letter to the editor
Geographic Code:7TURK
Date:Aug 1, 2010
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