Fast spontaneous recovery from acute necrotizing eosinophilic myopericarditis without need for immunosuppressive therapy: a case report of a 27-year-old male

Abstract

Background

Eosinophilic myocarditis (EM) is rare but accounts for 12–22% of histologically proven acute myocarditis cases. Acute necrotizing EM is considered an aggressive, life-threatening disease which is usually treated by high-dose corticosteroid therapy.

Case summary

We report the case of a 27-year-old man with acute severe pericarditic chest pain, moderately reduced left ventricular (LV) ejection fraction, and a small pericardial effusion. Troponin I level was highly elevated in the absence of coronary artery disease, leading to the diagnosis of acute myopericarditis. In the absence of blood eosinophilia and despite a negative cardiac magnetic resonance study, LV endomyocardial biopsy revealed an acute necrotizing EM. With conventional antiphlogistic and heart failure therapy, the patient became symptom-free and inflammatory and cardiac necrosis markers as well as LV ejection fraction normalized within days. Thus, in the absence of a systemic hypereosinophilic disorder, there was no need for steroid therapy. Long-term follow-up over 12 months showed sustained normalization of cardiac structure and function.

Discussion

Acute necrotizing eosinophilic myopericarditis is not always a dreadful cardiac disease. There are idiopathic cases which may quickly resolve without immunosuppression. There seems to be a publication bias towards critical cases.

Learning points

• Idiopathic acute necrotizing eosinophilic myopericarditis without blood eosinophilia may completely resolve under conventional guideline-directed medical therapy. In cases with rapid clinical and echocardiographic improvement, the omission of corticosteroid therapy may be considered.

• Cardiac magnetic resonance imaging for myocarditis may be negative despite biopsy-proven acute myocarditis.

Introduction

Eosinophilic myocarditis (EM) is considered to be a rare form of myocarditis, characterized by myocardial eosinophilic infiltration that is associated with blood eosinophilia in the majority of patients.^1–3 Eosinophilic myocarditis accounts for 12–22% of acute myocarditis cases.^4–6 Although an underlying systemic disorder can be identified in the majority of EM patients, one-third of EM cases are idiopathic.¹ Eosinophilic myocarditis without blood eosinophilia, which occurs in about 25% of histologically proven cases,¹ represents a particular diagnostic challenge. Acute necrotizing EM is considered an aggressive, life-threatening type of EM with a high death rate, which is usually treated by high-dose corticosteroid therapy.^7,8 Here, we report a young man with idiopathic acute necrotizing eosinophilic myopericarditis without blood eosinophilia who quickly recovered under standard heart failure medication without immunosuppression and showed normal cardiac function during a 12-month follow-up period.

Timeline

Timeline	Course of events
Day 0	Acute coronary syndrome-like presentation with systemic inflammatory response, moderately reduced ejection fraction, localized hypokinesia, and small pericardial effusion. Patient put on ibuprofene, colchicine, and heart failure medication
Day 1	Chest pain resolved, coronary artery disease excluded by angiography
Day 2	Cardiac magnetic resonance imaging showed small pericardial effusion and normalization of ejection fraction
Day 4	Endomyocardial biopsy result: acute necrotizing eosinophilic myocarditis
Day 5	Patient discharged from hospital without steroid therapy
Day 13	Patient free of symptoms, completely resolved inflammatory and myocardial necrosis markers, normal left ventricular (LV) function, still minimal localized pericardial effusion
2 months	Pericardial effusion completely resolved
6 months	Heart failure medication stopped, intense physical activities restarted
1 year	Patient free of symptoms, playing squash, normal LV systolic and diastolic function

Case presentation

A 27-year-old man presented to the emergency department at midnight with acute and ongoing mid-thoracic, oppressive chest pain, and increasing shortness of breath at rest. The chest pain worsened with inspiration. He also complained of a non-productive cough and excessive sweating in the last 2 days. One month prior to presentation in the hospital, he had returned from vacation in the Dominican Republic, where he was bitten by a lot of insects during a safari tour. He had no relevant medical or family history. He worked as an IT specialist and did not take any medication nor any illicit drugs.

We saw a heavily sweating, slightly overweight Caucasian male with a rapid pulse rate of 107 b.p.m. and a body temperature of 37.6°C. Breathing rate, blood pressure, oxygen saturation, and the remainder of the physical examination were all normal. The electrocardiogram (ECG) at admission showed sinus tachycardia with a discrete early repolarization pattern, but no changes typical for myocardial ischaemia or injury (Figure 1). Blood analysis revealed leucocytosis of 16000 cells/µL with a normal white blood cell differential distribution, particularly with a normal eosinophil count. The initial Troponin I (TnI) concentration was considerably elevated to 2.26 ng/mL (normal range < 0.02 ng/mL) and continued to rise to 7.11 ng/mL within 5 h. C-reactive protein (CRP) was increased to 114 mg/L (normal range 0–4 mg/L), but procalcitonin levels were normal. Serological markers for rheumatic diseases and vasculitis were all negative. Serum IgE concentration was normal. Chest X-ray showed a borderline normal heart size, no signs of pulmonary congestion nor pulmonary infiltrates nor pleural effusions. Emergency echocardiography revealed a moderately reduced left ventricular (LV) ejection fraction of 46% with basal to mid-inferolateral hypokinesia. The basal inferolateral segment also showed a tiny pericardial effusion with a maximum width of 4 mm (Figure 2). Another localized small pericardial effusion could be visualized by the subcostal view in front of the right ventricular wall. Left ventricular end-diastolic diameter was in the upper normal range (5.8 cm or 2.7 cm/m² BSA), LV wall thickness was normal, as well as cardiac valve morphology and function. There was no LV thrombus.

Figure 1.

Electrocardiogram on admission: sinus tachycardia (107 b.p.m.) and early repolarization pattern in leads V1–V3.

Figure 2.

Echocardiographic left parasternal long-axis view showing a subepicardial strip of increased echodensity (triangle) together with a tiny (4 mm) pericardial effusion (arrows) under the basal inferolateral segment of the left ventricle. Video sequences of the parasternal long- and short-axis are included in the Supplementary material online, Videos S1 and S2.

Under the working diagnosis of acute myopericarditis, the patient was transferred to our chest pain unit, where he received 5 mg of intravenous morphine and an anti-inflammatory combination of ibuprofen 600 mg t.i.d. together with colchicine 0.5 mg b.i.d. As further deterioration of LV function during the course of myopericarditis was feared, the patient was also treated with a heart failure medication consisting of a beta-blocker, an angiotensin-converting enzyme inhibitor, and a mineralocorticoid receptor antagonist. During the next hours, chest pain and dyspnoea completely resolved. Heart catheterization, which was performed at the next morning using a right transradial arterial access, revealed smooth coronary arteries (see Supplementary material online, Videos S3 and S4). Despite the relatively well preserved ejection fraction, LV end-diastolic pressure was considerably elevated to 29 mmHg (Figure 3). For endomyocardial biopsy (EMB), we took six specimens from the left ventricle. On the second day after admission, TnI had fallen to 1.34 ng/mL. Also, CRP had gone down to 33.7 mg/L without antibiotic therapy. The patient was transferred to the normal ward and cardiac magnetic resonance (CMR) imaging was performed, which showed a small pericardial effusion (Figure 4), but neither pericardial nor myocardial oedema and no late enhancement and a normal LV ejection fraction of 68% without regional wall motion abnormality. On the 4th day after admission—when patient’s state and his LV function already had recovered—the results from the myocardial biopsy arrived. These showed an acute necrotizing EM (Figure 5). In addition, parvovirus B19 (PVB19) DNA with a moderately increased viral load of 1364 copies/µg of isolated nucleic acids and Epstein–Barr virus DNA with a low copy number were detected. PVB19 mRNA intermediates indicating active replication could not be detected. Other PCRs for cardiotropic viruses or bacteria were all negative (see Supplementary material online, Supplement) .

Figure 3.

Left ventricular pressure tracing at catheterization (8 h after admission). Despite an only moderately impaired ejection fraction of 46%, left ventricular end-diastolic pressure was increased to 29 mmHg.

Figure 4.

Four-chamber view depicted by fast imaging employing steady-state acquisition (FIESTA) cardiac magnetic resonance imaging. A small pericardial effusion is seen in front of the right ventricle and a tiny one at the basal lateral wall of the left ventricle. Additional cardiac magnetic resonance sequences and pictures are included in the Supplementary material online, Videos S5–S8, Images S9–S11.

Figure 5.

Left ventricular endomyocardial biopsies reveal myocyte necrosis (arrows) and CD3+ T cells (brown) (A) and eosinophilic granulocytes (arrows in (B), Giemsa). Original magnification ×200 (A), ×400 (B).

On the 5th day after admission, concentrations of TnI and CRP had dropped down to 0.04 ng/mL, and 8.9 mg/L, respectively, and the patient was discharged from hospital.

Eight days after discharge, the patient continued to be free of symptoms. The ECG showed normal sinus rhythm (59 b.p.m.), an unchanged early repolarization pattern and an incomplete right bundle branch block. Leucocyte and eosinophil count, CRP, brain natriuretic peptide, TnI were all normal. On echocardiography, there still was a minimal residual pericardial effusion gap of 3 mm. Left ventricular end-diastolic diameter was normal, as was LV ejection fraction (67%; see also Supplementary material online, Videos S12 and S13) as well as LV diastolic function. Left ventricular filling index (E/E´) was 7.0 indicating a normalization of the initially increased LV filling pressure. Ibuprofen was stopped, colchicine was stopped 2 months later. The patient was advised to refrain from high-intense physical activity for the following 6 months.

Echocardiography at 2, 6, and 12 months after discharge showed normal systolic and diastolic LV function. The residual pericardial effusion had completely resolved at 2 months. At 6 months, heart failure medication was tapered and the patient restarted leisure sports activities.

Discussion

Though acute myocarditis may be diagnosed non-invasively by CMR imaging with acceptable diagnostic accuracy^3,9 our case underlines that even in biopsy-proven myocarditis, CMR might be negative or non-diagnostic.¹⁰ Endomyocardial biopsy confirms the suspicion of myocarditis and identifies the underlying aetiology and the type of inflammation, which may imply different treatment strategies.¹¹ Among those causes of myocarditis that usually require immunosuppression, acute necrotizing EM, which is far less common than lymphocytic myocarditis but probably more frequent than giant cell myocarditis,^4,6 is considered to be a life-threatening disease,^1,3,7,8 for which an in hospital mortality of 22% was reported in a recent systematic review of the literature.¹

The case presented here is remarkable, because it demonstrates that there are courses of idiopathic acute necrotizing EM without blood eosinophilia, which quickly recover completely without immunosuppressive therapy. As acute necrotizing EM is rare and most literature cases report critically ill patients who are treated with high-dose corticosteroids,^1,2,7,8 it can be assumed that there is a publication bias which leads to overestimation of both, the mortality of this disease entity as well as the need for immunosuppressive therapy. Interestingly, a retrospective observation from Japan reported 22 cases of idiopathic EM, who were treated without steroids and had a zero mortality rate after 1 year.¹² Hence, at least in idiopathic cases of acute necrotizing EM without blood eosinophilia who exhibit only a slightly to moderately impaired LV function and who experience a rapid recovery with conventional therapy, it seems justified to withhold immunosuppressive therapy. This is all the more important, when, as in our case, viral genomes are detected by EMB, which usually precludes immunosuppression.¹¹

Lead author biography

Prof. Michael Kindermann is head of the Department of Cardiology, Angiology and Pneumology of the CaritasKlinikum St. Theresia in Saarbrücken, Germany, which is an academic teaching hospital of the Saarland University. His main clinical and research interests are interventional cardiology and angiology, myocardial disease, echocardiography, and heart failure management.

Supplementary material

Supplementary material is available at European Heart Journal - Case Reports online.