This 36-year-old male patient, at the age of 22 years, had had painless scalp swelling due to multiple aneurysms of the left temporal artery. Biopsy revealed an eosinophilic infiltration of all three wall layers with fresh fibrinoid necrosis beside old fibrous scars. An idiopathic blood eosinophilia was noted; no treatment was initiated.
In the present case, the 36-year-old patient presented with dyspnea at rest. The ECG showed evidence of prior inferior infarction. Transesophageal echocardiography found left ventricular (LV) enlargement with biplanar ejection fraction of 20% as well as dilatation of the right ventricle and the atria. LV muscle mass was increased (245 g/m2; normal, 134 g/m2). The epicardial coronary arteries showed grotesque aneurysmal dilatation. Intravascular thrombosis of the left and right main stem with reticular revascularization could be clearly identified (Fig 1⇓). Ultrasonic myocardial texture was normal, without signs of focal granulomatosis, whereas endocardial echo density was enhanced, indicating endocardial fibrosis. Right ventricular endomyocardial biopsy yielded interstitial scars in the myocardium. However, there was no infiltration with eosinophilic leukocytes. Angiography visualized proximal obliteration of the left anterior descending and right coronary arteries. The left circumflex artery was subtotally obliterated (Figs 2⇓ and 3⇓). Aortography revealed a normal abdominal aorta and intestinal arteries but a stenosis of the arteria hepatica propria. The small vessels of the inferior spleen were not patent. Laboratory tests found hypereosinophilia (48%) and elevated IgE. There was no evidence of parasitosis, atopic disease (eg, asthma), or neoplastic or connective tissue disease.
Persistent eosinophilia from various causes may be associated with the so-called hypereosinophilic syndrome, comprising endomyocardial fibrosis, valvular lesions, mural thrombus formation, cardiomegaly due to infiltration of the myocardium with eosinophils, and pericardial effusion.1 2 3 Furthermore, the patients have asthma and gastrointestinal and nervous system dysfunction. In contrast, the patient presented here has no signs of widespread infiltration of organs with eosinophils but shows systemic vasculitis with eosinophilia and fibrinoid necrosis, leading to aneurysm formation. The clinical picture is distinct from Churg-Strauss syndrome, periarteritis nodosa, and juvenile arteritis temporalis, all of which are also occasionally described to manifest at the coronary vessels and to be associated with blood eosinophilia.4 5 However, precise nosological classification of the systemic eosinophilic vasculitis in our patient is not possible because of absence of organ involvement and negative tests for autoantibodies, eg, anti-neutrophil cytoplasmic antibodies.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Lukes Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner, MC 4-265, Houston, TX 77030.
- Copyright © 1995 by American Heart Association
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