Iran Red Crescent Med J. 2015 November; 18(11):e28697. doi: 10.5812/ircmj.28697.

Published online 2015 August 10. Case Report Dressler Syndrome: A Case Report

Thiago Andrade Macedo,1,* Roberto Nery Dantas Junior,2 Pedro Gabriel Melo de Barros e Silva,1 and

Marcio Campos Sampaio3

1Cardiology Division, Institute (InCor), Medical School of University of Sao Paulo (FMUSP), Sao Paulo, Brazil 2Cardiovascular MRI and CT Division, Heart Institute (InCor), Medical School, University of Sao Paulo (FMUSP), Sao Paulo, Brazil 3Cardiovascular MRI and CT Division, Institute Dante Pazzanese of (IDPC), Sao Paulo, Brazil

*Corresponding author: Thiago Andrade Macedo, Cardiology Division, Heart Institute (InCor), Av Dr Eneas Carvalho de Aguiar, 44, Zip Code: 05403-904, Sao Paulo, Brazil. Tel: +55-1135677586, Fax: +55-1126615948, E-mail: [email protected]

Received 2015 March 29; Revised 2015 June 13; Accepted 2015 July 14.

Abstract

Introduction: Dressler Syndrome should be considered in the differential diagnosis of , especially in patients who are in a late stage of the evolution of the ischemic process. Case Presentation: A 46-year-old male was admitted to the emergency department due to pleuritic chest pain. Two weeks before this admission, he presented with a typical episode, likely an ST segment elevation (STEMI), and did not receive reperfusion therapy or any medical care. The patient’s electrocardiogram showed diffuse ST segment elevation and PR segment depression, and his blood tests showed positive myocardial necrosis markers. A coronary angiography showed a proximal occlusion (not recanalized) of the circumflex artery. There was a late gadolinium enhancement area seen through cardiac magnetic resonance imaging (CMR), suggestive of recent transmural infarction, pericardial injury, and pleural effusion (inflammatory). Conclusions: These findings strongly suggest the diagnosis of delayed post-infarction , or Dressler Syndrome, a rare disease in the age of reperfusion therapy. Although rare, it is a syndrome that must be considered in the differential diagnosis of chest pain.

Keywords: Chest Pain, Myocardial Infarction, Pericarditis, Dressler Syndrome

1. Introduction during physical exertion, radiating to the jaw, but did not seek medical care. Two weeks later, the patient came Dressler syndrome (DS) was first described by William to the emergency department of the heart institute (In- Dressler in 1956, after observing the late period devel- Cor) at the University of Sao Paulo, Brazil, in January of opment of an acute myocardial infarction (1). It is an 2015, reporting pain of a different pattern: severe con- immuno-inflammatory disease similar to others that oc- strictive chest pain lasting one hour that worsened with cur after myocardial injury, such as post-pericardiectomy deep inspiration and while in a supine position. The phys- syndrome and post-traumatic pericarditis (2). After my- ical examination demonstrated a blood pressure of 140/85 ocardial infarction (MI), injuries to the cardiac myocytes mmHg (semi-automatic digital sphygmomanometer, Om- expose myocardial antigens to the immune system, stimu- ron 705CP, calibrated), (105 bpm), tempera- lating immune complex formation. This immune system ture of 99.5°F, and no jugular venous distension. A peri- activation triggers a systemic immune-inflammatory re- cardial friction sound was easily audible at the lower left sponse that may involve other organs, such as the pleura, sternal border. Moreover, the electrocardiogram (EKG Ma- due to immune cross-reactions (3). The symptoms tend to chine, Philips PageWriter Trim III, calibrated) showed dif- occur 2 - 3 weeks after MI, and may include pleuritic chest fuse ST-segment elevation with a depression of the PR seg- pain and . A direct relationship between systemic ment, mainly in DII (Figure 1), highly suggestive of peri- inflammation and myocardial has been demon- . Chest radiography showed a cardiac area in the strated by several authors (2,3). upper normal limit, and mild right pleural effusion. The results of the laboratory blood tests were as follows: glu- 2. Case Presentation cose = 307 mg/dl, CKMB = 5.8 ng/mL (normal = < 4 ng/mL), troponin I = 18 ng/mL (normal = < 1.0 ng/mL), Hb = 15.2 g/l, A 46-year-old male with hypertension and diabetes had WBC = 8,600/µL (no predominance of younger lineages), a 30-minute long episode of strong oppressive chest pain

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Table 1. Patient’s Characteristics received full anti-ischemic drug treatment, in addition to aspirin at an anti-inflammatory dose of 2 g/day, and ex-

Variables Results hibited improvement in his chest pain. He was then dis- charged on the fourth day of hospitalization. Age in years 46

Gender Male

Symptoms Chest pain worsening with deep inspiration 3. Discussion

Pulse rate, bpm 105

Blood pressure, mmHg 140/85 A young patient (46-year-old) with hypertension and diabetes was admitted to the emergency department com- Cardiac auscultation Pericardial friction easily audible plaining of chest pain. The presence of constrictive chest µ WBC, L 8,600 pain in a patient with risk factors CKMB, ng/mL 5.8 initially suggested acute myocardial ischemia. However, Troponin I, ng/mL 18 the worsening of the chest pain upon deep breathing and

ECG Diffuse ST-segment elevation and depression of while in a supine position, in addition to a clear pericar- the PR segment dial friction sound, could also indicate pericarditis. In the X-ray radiography Cardiac area in the upper normal limit clinical history, the patient had an initial complaint of typ-

LVEF 0.34 ical angina two weeks previously, followed by the actual pleuritic chest pain which led him to our emergency de- CMR Pericardial injury and pleural effusion (inflammatory) partment. The electrocardiogram, demonstrating diffuse ST-segment elevation and depression of the PR, was highly Abbreviations: CMR, cardiac magnetic resonance imaging; ECG, electrocardio- gram; LVEF, left ventricle ejection fraction; WBC, white blood cell. suggestive of pericarditis. In some cases, PR segment de- pression can occur even in the absence of ST-segment eleva- tion, and may appear as the initial manifestation of acute and platelets = 162,000. The lipid profile was normal, the pericarditis (4). myocardial necrosis markers decreased progressively dur- The elevation of the myocardial necrosis markers may ing an in-hospital stay, and the echocardiogram (GE Vivid indicate coronary ischemia or the myopericardial patho- 7) demonstrated inferolateral hypokinesia, with a left ven- logical process known as . Patients with tricle ejection fraction (LVEF) of 0.55, without pericardial who develop great elevations effusion. The clinical characteristics are described in Table in their myocardial necrosis markers, especially without 1. reperfusion therapy (as in this case), are at a higher risk of The coronary angiography revealed proximal occlu- developing post-infarction pericarditis (5). sion of the circumflex artery, without other stenosis, and Interestingly, the markers collected upon admission an angioplasty attempt was unsuccessful due to the im- showed the typical kinetics of a subacute process of about possibility of overcoming the obstruction. Cardiac mag- 2 weeks: the CKMB was almost normal (5.8 ng/mL) and netic resonance imaging (CMR) was performed to analyze the troponin, although still very high (18 ng/mL), was also the , and to better characterize the extent of reducing progressively. The echocardiogram showed no the ischemia. A transmural inferolateral late gadolinium evidence of pericardial effusion, which is not mandatory enhancement was found, suggestive of fibrosis due to my- for the diagnosis of pericarditis. However, the evidence of ocardial infarction (MI) (Figure 2A), with an underlying persistent chest pain, elevated myocardial necrosis blood area of no-reflow (microvascular obstruction, indicating a markers, and inferolateral hypokinesia without pericar- recent injury), inferior akinesia (LVEF = 0.34), pericardial dial abnormalities, led to an early coronary angiography, injury (a small area of epicardial scarring in the left lateral which detected a proximal occlusion of the circumflex wall), and mild right pleural effusion (Figure 2B). A semiau- artery without the features of acute thrombosis. The sus- tomatic quantification of fibrosis determined a 7.6 g my- picion of persistent ischemia motivated an attempt to per- ocardial scar (8% of the total myocardial volume) by that form angioplasty, which was not possible due to the inabil- time (3 standard deviations and full width at half maxi- ity to overcome the obstruction, reinforcing the hypoth- mum, Circle CVI Software, version 4.2.1). esis of a chronic or subacute injury to that coronary site. The clinical features of the patient’s chest pain (differ- The patient underwent a CMR to further determine the ent from the initial pain two weeks before), and the highly ischemia burden, and for a better evaluation of the peri- suggestive electrocardiogram and CMR findings suggest cardium (6). Confirming the clinical hypothesis, the CMR late post MI pericarditis or Dressler Syndrome. The patient images showed inferolateral transmural necrosis (with

2 Iran Red Crescent Med J. 2015; 18(11):e28697. Macedo TA et al.

Figure 1. Electrocardiogram Showing Diffuse ST Segment Elevation with Depression of the PR Segment, Mainly in DII

Figure 2. CMR images demonstrating: A, left ventricle inferolateral transmural fibrosis in white (arrow) and pericardial damage*; and B, late gadolinium enhancement sequences (four chamber) with a small pleural effusion (arrow) (cine SSFP four chamber image); CMR: cardiac magnetic resonance imaging, SSFP: steady state free precession. features of subacute evolution), subtle pericardial involve- (Figure 2B). The patient’s history, laboratory, and imag- ment (but with no effusion), and evident right pleural ef- ing findings led to the hypothesis of an acute MI without fusion, which is an inflammatory signal described in DS reperfusion therapy two weeks before his visit to the emer-

Iran Red Crescent Med J. 2015; 18(11):e28697. 3 Macedo TA et al.

gency department, which was complicated by late peri- Footnote carditis, strongly suggesting the possibility of DS. In the case described, the immuno-inflammatory response was Authors’ Contribution: Study concept and design: Thi- suggested by the presence of pleural effusion, similar to ago Andrade Macedo; acquisition of data: Roberto Nery other descriptions of the clinical presentation of DS (7), Dantas Junior; analysis and interpretation of data: Pedro correctly evidenced by the CMR (Figure 2B). Gabriel Melo de Barros e Silva and Marcio Campos Sam- There was an interval of two weeks between the typi- paio; drafting of the manuscript: Thiago Andrade Macedo; cal angina (probably related to the MI) and the new chest critical revision of the manuscript for important intel- pain with characteristics of pericardial involvement. The lectual content: Thiago Andrade Macedo; administrative, diagnosis of DS requires the occurrence of pericardial in- technical, and material support: Pedro Gabriel Melo de Bar- jury in the subacute post-MI period. In a study of 15 cases ros e Silva and Marcio Campos Sampaio; study supervision: with characteristics of DS, the clinical presentation of pleu- Thiago Andrade Macedo. ritic pain occurred up to the 21st day after the MI in 66.7% of the patients (8). References Before the era of reperfusion, DS occurred after about 1% - 5% of the reported MIs; however, the incidence of DS 1. Dressler W. A post-myocardial infarction syndrome; preliminary re- has declined dramatically, either after the advent of fibri- port of a complication resembling idiopathic, recurrent, benign peri- carditis. J Am Med Assoc. 1956;160(16):1379–83. [PubMed: 13306560]. nolysis (9) or after the spread of percutaneous reperfusion 2. Versey JM, Gabriel R. Soluble-complex formation after myocardial in- treatment. A prospective study of 201 patients with acute farction. Lancet. 1974;2(7879):493–4. [PubMed: 4137193]. MIs undergoing thrombolytic therapy did not detect the 3. Neumann FJ, Ott I, Gawaz M, Richardt G, Holzapfel H, Jochum M, onset of DS in any patient undergoing early reperfusion (5). et al. Cardiac release of cytokines and inflammatory responses in acute myocardial infarction. Circulation. 1995;92(4):748–55. [PubMed: Based on this finding, it was postulated that the decrease 7543831]. in the infarct size promoted by the early coronary reperfu- 4. Baljepally R, Spodick DH. PR-segment deviation as the initial sion, and the shorter exposure of the immune system to electrocardiographic response in . Am J Cardiol. the myocardial antigens, promoted the virtual disappear- 1998;81(12):1505–6. [PubMed: 9645908]. 5. Bonnefoy E, Godon P, Kirkorian G, Fatemi M, Chevalier P, Touboul ance of this entity (10, 11). P. Serum cardiac troponin I and ST-segment elevation in pa- This case highlights the importance of the clinical as- tients with acute pericarditis. Eur Heart J. 2000;21(10):832–6. doi: sessment of patients admitted with chest pain. Although 10.1053/euhj.1999.1907. [PubMed: 10781355]. rare, Dressler Syndrome should be considered in the dif- 6. Lawley C, Mazhar J, Grieve SM, Figtree GA. Visualizing pericar- dial inflammation in Dressler’s syndrome with cardiac mag- ferential diagnosis of chest pain, especially in patients who netic resonance imaging. Int J Cardiol. 2013;168(1):32–3. doi: are in a late stage of the evolution of the ischemic process. 10.1016/j.ijcard.2013.05.082. [PubMed: 23769683]. This becomes especially important in developing coun- 7. Goossens K, Caenepeel A, De Greef Y. Delayed tamponade trigger- ing Dressler’s syndrome after pulmonary vein isolation. Acta Cardiol. tries, where many patients receive no reperfusion therapy 2012;67(5):595–8. doi: 10.2143/AC.67.5.2174137. [PubMed: 23252013]. due to difficulties in accessing the healthcare system. 8. Sahasranam KV, Chandra P, Ravindran KN. Early onset Dressler’s syndrome–a study of fifteen cases. Indian J Chest Dis Allied Sci. 1990;32(3):153–6. [PubMed: 2081629]. Acknowledgments 9. Shahar A, Hod H, Barabash GM, Kaplinsky E, Motro M. Disappearance of a syndrome: Dressler’s syndrome in the era of thrombolysis. Cardi- The authors would like to thank Valeria Costa Hong for ology. 1994;85(3-4):255–8. [PubMed: 7987883]. her guidance in the organization of the references. 10. Bendjelid K, Pugin J. Is Dressler syndrome dead?. Chest. 2004;126(5):1680–2. doi: 10.1378/chest.126.5.1680. [PubMed: 15539743]. 11. Northcote RJ, Hutchison SJ, McGuinness JB. Evidence for the con- tinued existence of the postmyocardial infarction (Dressler’s) syn- drome. Am J Cardiol. 1984;53(8):1201–2. [PubMed: 6702705].

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