Eosinophilic Myocarditis Demonstrated Using Cardiac Magnetic Resonance Imaging in a Patient with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Disease)

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), historically known as the Churg-Strauss disease, is a small- to medium-sized vessel multi-organ vasculitis with a propensity to involve the heart. EGPA is a rare condition with an estimated annual incidence of one to 4.2 people per million. The cardiac involvement causes significant morbidity and mortality in EGPA patients. Approximately 50% of the deaths in EGPA are related to cardiac disease and occur within the first few months since diagnosis. The current recommendations support evaluation of cardiac involvement by using history, physical exam and multimodality imaging including echocardiogram and cardiac magnetic resonance imaging (CMR). Here, we report a rare case of eosinophilic myocarditis in a 19-year-old patient with EGPA seen on CMR. Pertinent literature is also reviewed. We highlighted the importance of CMR in diagnosing and follow up of EGPA patients.

Keywords: cardiac magnetic resonance imaging; churg strauss disease; coronary cta; egpa; elevated troponins; eosinophilic myocarditis; five factor score.

Figure 1. Electrocardiogram.

(A) Initial electrocardiogram (ECG) showed atrial fibrillation with a heart rate of 161, non-diagnostic Q waves in the inferior leads, T-wave inversions in the inferior leads and no significant ST segment changes noted. (B) Repeat ECG 10 minutes later showed sinus tachycardia with a heart rate of 100, nondiagnostic Q waves in the inferior leads and T-wave inversions in the inferior leads as well and no significant ST segment changes noted.

Figure 2. Computed tomography angiography (CTA) chest.

CTA of the chest: Bilateral patchy ground-glass opacities (blue arrow), greatest at the posterior lower lobes. Some additional more nodular appearing opacities (red arrow) are present and also greater in the lower lobes.

Figure 3. Cardiac magnetic resonance.

(A, B) Cardiac magnetic resonance triple inversion T2 weighted image showing edema of the left ventricle wall (blue arrow heads).

Figure 4. Cardiac magnetic resonance.

(A, B) Cardiac magnetic resonance short axis view showing patchy areas of the delayed myocardial enhancement of the Gadolinium within the left ventricular myocardium (blue arrow head). The abnormal delayed myocardial enhancement pattern is most consistent with myocarditis. There is fairly discrete region of decreased perfusion in the mid to apical septal and inferior segments and throughout the apex (red arrow heads). Segmental perfusion abnormality on dynamic imaging may be the result of hypoperfusion associated with vasculitis.

Figure 5. Pathology images of cryobiopsies.

(A) 20x image. Microscopic examination demonstrates dense eosinophilic infiltrate involving alveolated lung parenchyma and many small venules, accompanied by patchy organization (black arrow) (H&E stained sections, 20x). (B) 40x image. Eosinophilic venulitis. Chronic inflammation including many eosinophils infiltrating venular wall (black arrow head) (H&E stained sections, 40x).