Abstract

Eosinophilic Myocarditis is an acute life-threatening inflammatory disease of the heart. It is often an elusive diagnosis and treatment is straightforward. Very few cases of eosinophilic myocarditis have been reported.

Case Report

A 33 years old male with no comorbidities presented at our emergency department with a history of abdominal pain, epigastric region, colicky in character, non-radiating, aggravated on changing posture, relieved on taking rest for the past seven days. He had no history of fever with chills or rigors, no history of any rashes, cough, cold, vomiting, loose stools, decreased urine output, palpitations, breathlessness, headache, backpain, excessive sweating or giddiness. He is neither a smoker nor consumed alcohol. He has not taken any medications for the above complaints. No history of any allergy in the past.

On receiving him in the Emergency Department he was conscious, oriented, afebrile with a blood pressure of 110/70mmhg, pulse:105 beats/min, respiration of 20/min and a temperature of 98.4°F.He had no pallor, icterus, cyanosis, clubbing, lymphadenopathy or pedal edema. On systemic examination, the chest was clear with bilateral air entry with no added sounds. Cardiac: S1S2 heard, no murmur. Abdomen revealed diffuse abdominal tenderness predominant in the epigastric region, right hypochondrium and umbilical region with guarding rigidity. Bowel sounds were heard. Per rectum: soft stools, no bloodstains or fissure. Routine investigations were done.

Investigation and management:

CT Chest:Mild right-sided pleural effusion with bilateral basal dependent atelectatic changes. Cardiomegaly.

CT Abdomen: The gall bladder is moderately distended with mild diffuse edematous wall thickening. Mild inflammation along the second part of the duodenum, right paracolic gutter with mild thickening of right anterior and pararenal and lateral conal fascia.

ECHO demonstrated: Global hypokinesia of Left ventricular
Severe Left ventricular Systolic Dysfunction (LVEF=30%)
Grade II AR, Mild MR
Grade II TR (RVSP-41mmHg)
Tachycardia noted during study (HR:103)
Dilated IVC -Non collapsing
ECG showing heart rate:100/min, sinus rhythm, normal axis, normal interval, no significant st/t changes

Because of persisting pain, he was administered with proton pump inhibitor and antiemetic. In view of EF:30% and heart rate:103 and poor ejection fraction, he was shifted to the coronary care unit. Cardiology opinion was obtained- advised Trop I:Negative and ECG showed no abnormal waves. Loading doses given as prophylaxis (tab.clopilet, tab.aspirin, tab.aztor, tab.flavedon mr and inj.lasix20mg). A gastroenterologist’s opinion was obtained that suggested possibilities of eosinophilic enteritis n colitis. Advised to do a mucosal and myocardial biopsy, for further evaluation but the patient deferred.

Although no controlled trial data or guidelines exist, observational cases suggest a benefit from early immunosuppression with agents such as corticosteroids, cyclophosphamide, intravenous immunoglobulin, or antithymocyte globulin. Recently, Brambatti et.al. found a lower incidence of in-hospital death among those treated with corticosteroids (9.9%) versus those who were not (65.7%)[5]

Later started on corticosteroid injection and fluid restriction <1litre /day. Repeated CBC showed an increasing trend of eosinophil Count, hence the provisional diagnosis of eosinophilic myocarditis. The patient was maintained in progressively lower doses of corticosteroid, he felt symptomatically better and so was discharged with tablet wysolone 15mg od and was requested to review after one month.

The patient returned for follow-up, he was asymptomatic and hemodynamically stable.

Discussion

It is an inflammation in the heart muscle that is caused by the infiltration and destructive activity of a type of white blood cell, the eosinophil. Research has been more focused on eosinophilic heart diseasesince1936 when Löffler described two cases of endocarditis parietalis fibroplastica [1]. It was identified in 0.5% of cases in an unselected autopsy series of patients with heart failure and 0.1% of cases among a cohort of patients biopsied for suspected myocarditis. The prevalence of Eosinophilic myocardtitis in patients undergoing heart transplantation has been reported to be 3% to 7%.[2,3]

Eosinophilia is considered to be reactive in the lung, GIT, and urogenital tumors as well as in certain types of hematologic disorders such as T-cell and Hodgkin lymphomas.

There are three phases of eosinophilic heart disease:

Typically the disorder is associated with hyper eosinophilia with eosinophil count >1500/microlitre (normal range 100-400). Other types include reactive eosinophilia caused by various microbial agents which includes [7]

Symptoms of the disorder are the same as those seen in other forms of heart disease: chest pain, shortness of breath, fatigue, chest palpitations, lightheadedness, and syncope. In these syndromes eosinophilia may arise from medications, hypersensitivity disorders, metastatic malignancies, endocrinopathy, autoimmune disorders, or leukemia; however, some cases remain idiopathic.[1] Endomyocardial biopsy is the gold standard for the diagnosis of EM; however, the sensitivity is only 54%, cardiac magnetic resonance is the gold standard in the non-invasivediagnosis of myocarditis. The use of ECG, echocardiography, myocardial biopsy and cardiac biomarkers aid in the diagnosis.[10]

Take-home points

1.Diagnosis of Eosinophilic myocarditis is usually made when the patient has
• eosinophilia (>0.5 K/mm3);
• symptoms of chest pain, dyspnea, or palpitations;
• elevated cardiac biomarkers;
• ECG changes, particularly ST-segment elevation (≈50% of cases) and pathological Q waves (≈20% of cases); and
• LV wall thickening and abnormal wall motion on echocardiography.[11]
2.Peripheral blood eosinophilia associated with cardiac symptomatology should always raise suspicion for Eosinophilic myocardtitis. It is often a forgotten cause, hopefully not anymore.

Acknowledgments

I would like to thank,
Dr. S.Vadivel Kumaran (Medical Gastroenterologist)
Dr. K.P.Suresh Kumar (Senior Consultant Cardiologist), Dr. Vishnu (Consultant Cardiologist),
Dr. Aslesha Vijaay Sheth (Head of Emergency Department)
Dr. Vidya (Emergency Physician)
for guiding me with the article.

Review of Literature

1. Oakley C. M., Olsen E. G. J. Eosinophilia and heart disease. British Heart Journal. 1977;39(3):233–237. Doi: 10.1136/hrt.39.3.233.
2. Ali AM, Straatman LP, Allard MF, Ignaszewski AP. Eosinophilic myocarditis: case series and review of literature.Can J Cardiol. 2006; 22:1233–1237.
3. S, Sugiyama Kato T, Mancini D, C Marboe C. Characteristics of patients with advanced heart failure having eosinophilic infiltration of the myocardium in the recent era.Int Heart J. 2013; 54:146–148. doi: 10.1536/ihj.54.146.
4. Working Group. Guidelines for diagnosis and treatment of myocarditis (JCS 2009): digest version.Circ J. 2011; 75:734–743. doi: 10.1253/circj.CJ-88-0008.
5.Brambatti M, Matassini MV, Adler ED, Klingel K, Camici PG, Ammirati E. Eosinophilic myocarditis: characteristics, treatment, and outcomes.J Am Coll Cardiol. 2017; 70:2363–2375. doi: 10.1016/j.jacc.2017.09.023.
6. Cugno M., Marzano A. V., Lorini M., Carbonelli V., Tedeschi A. Enhanced tissue factor expression by blood eosinophils from patients with hypereosinophilia: a possible link with thrombosis. PLoS ONE. 2014;9(11) doi: 10.1371/journal.pone.0111862.e111862.
7.Baandrup U. Eosinophilic myocarditis. Herz. 2012;37(8):849–853. Doi: 10.1007/s00059-012-3701-2.
8.Masi A. T., Hunder G. G., Lie J. T., et al. The American college of rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis) Arthritis and Rheumatism. 1990;33(8):1094–1100
9.Ogbogu P. U., Bochner B. S., Butterfield J. H., et al. Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. Journal of Allergy and Clinical Immunology. 2009;124(6):1319.e3–1325.e3. doi: 10.1016/j.jaci.2009.09.022
10. Friedrich M. G., Sechtem U., Schulz-Menger J., et al. Cardiovascular magnetic resonance in myocarditis: a JACC white paper. Journal of the American College of Cardiology. 2009;53(17):1475–1487. Doi: 10.1016/j.jacc.2009.02.007.
11. Caforio A. L. P., Pankuweit S., Arbustini E., et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. European Heart Journal. 2013;34(33):2636–2648. Doi: 10.1093/eurheartj/eht210.

Dr. Mohammed Rashid
MRCEM Resident – Department of Emergency Medicine