The cardiovascular manifestations of the hypereosinophilic syndrome: Prospective study of 26 patients, with review of the literature

doi:10.1016/0002-9343(79)90227-4

The American Journal of Medicine

Volume 67, Issue 4, October 1979, Pages 572-582

https://doi.org/10.1016/0002-9343(79)90227-4 Get rights and content

Abstract

The major cause of morbidity and mortality in patients with the hypereosinophilic syndrome is cardiac dysfunction. A review of 65 cases from the literature (historic series) revealed the following cardiovascular manifestations to be most common: dyspnea (60 per cent), signs of congestive heart failure (75 per cent), murmur of mitral regurgitation (49 per cent), cardiomegaly (37 per cent), T wave inversions on electrocardiogram (37 per cent) and pathologic findings of endocardial fibrosis, myocardial inflammation and mural thrombus formation (57 per cent). We have prospectively followed 26 patients with the hypereosinophilic syndrome for up to nine years (average follow-up prospectively was 3.3 years, retrospectively 5.7 years). Common cardiac findings in our 26 patients were dyspnea (42 per cent), chest pain (27 per cent), signs of congestive heart failure (38 per cent), murmur of mitral regurgitation (42 per cent), cardiomegaly (35 per cent) and T wave inversions (35 per cent). Thus, these patients demonstrated cardiovascular manifestations similar to those in the historic series, although the literature review showed a higher incidence of overt congestive heart failure.

Of 22 patients having echocardiograms, 55 per cent demonstrated some clinical, roentgenographic or electrocardiographic evidence of cardiac involvement, but 82 per cent had echocardiographic abnormalities. This suggests that the echocardiogram is a sensitive and perhaps early indicator of cardiac involvement in this disease. Common echocardiographic findings included increased left ventricular wall thickness (68 per cent), left ventricular mass (73 per cent) and left atrial size (37 per cent). Prospective echocardiographic follow-up of 18 patients (for up to four and a half years) revealed that seven of eight untreated or inadequately treated patients had increases in left ventricular wall thickness, whereas all 10 adequately treated patients had decreases (eight of 10) or no change (two of 10) in left ventricular wall thickness. This suggests that adequate antihypereosinophilic therapy (with prednisone and/or hydroxyurea) may stabilize and, in some cases, reverse the cardiac manifestations of the hypereosinophilic syndrome.

In previous studies, congestive heart failure due to eosinophilic cardiomyopathy has been reported to be very resistant to therapy. In our patients with congestive heart failure, treatment has been almost invariably effective when digitalis and diuretics were combined with adequate antihypereosinophilic therapy.

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Hypereosinophilic syndrome is a progressive disease with extensive eosinophilia that results in organ damage. Cardiac pathologies are the main reason for its high mortality rate. A better understanding of the mechanisms of eosinophil-mediated tissue damage would benefit therapeutic development. Here, we describe the cardiac pathologies that developed in a mouse model of hypereosinophilic syndrome. These IL-5 transgenic mice exhibited decreased left ventricular function at a young age which worsened with age. Mechanistically, we demonstrated infiltration of activated eosinophils into the heart tissue that led to an inflammatory environment. Gene expression signatures showed tissue damage as well as repair and remodeling processes. Cardiomyocytes from IL-5Tg mice exhibited significantly reduced contractility relative to wild type (WT) controls. This impairment may result from the inflammatory stress experienced by the cardiomyocytes and suggest that dysregulation of contractility and Ca²⁺ reuptake in cardiomyocytes contributes to cardiac dysfunction at the whole organ level in hypereosinophilic mice.
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Citation Excerpt :
In the second stage, the damaged endocardium is replaced by a thick layer of granulation tissue with concomitant activation of procoagulant factors, leading to the thrombosis. In a prospective cohort of 26 patients with HES, endomyocardial thickening was detected in 68% of cases and was progressive in untreated or inadequately treated patients.20 The presence of endomyocardial thickening by echocardiography in the setting of HES should prompt suspicion of LE.
Loeffler's endocarditis (LE) is the cardiac manifestation of hypereosinophilic syndrome, a rare systemic disease characterized by the sustained production of eosinophils leading to organ damage. Few data, principally by case reports, are available regarding the diagnostic workup in patients with suspected LE. Thus, we have performed a systematic search of the literature dealing with imaging in LE and propose an integrated multimodality imaging approach in the cardiac diagnostics of LE patients. The aim is to provide an updated state-of-the-art review focused on noninvasive and invasive imaging modalities for this rare and underdiagnosed disease. Standard and advanced echocardiography are typically the first cardiac imaging examinations when LE is suspected and they are also used later in follow-up for prognostic stratification and assessing response to treatment. Cardiac magnetic resonance provides a more detailed anatomical and functional evaluation of cardiac chambers, tissue characterization for the presence and extension of myocardial edema and fibrosis, and ventricular thrombi identification. Computed tomography scan and [18F]-fluoro-deoxy-glucose positron emission tomography may be helpful in selected cases to evaluate the cardiac involvement of LE as well as the other noncardiac manifestations of hypereosinophilic syndrome. Endomyocardial biopsy may be considered in patients with high clinical suspicion of LE if noninvasive imaging findings are confusing or not conclusive. The appropriate use of invasive and noninvasive imaging modalities, combining the available techniques with the patients' clinical features, will hopefully lead to early diagnosis, more accurate staging of disease, and timely treatment of LE that may prevent the irreversible myocardial damage of LE and adverse cardiovascular events.
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We present a 60-year-old patient with a history of recurrent infective endocarditis due to different Streptococcus species. Transthoracic echocardiography (TTE) revealed a circumscript echodense structure near the apex of the left ventricle, suggestive of a vegetation. Positron emission tomography revealed increased activity at the same site of the left ventricle and showed no other abnormalities. The patient was treated with intravenous antimicrobial therapy for each episode of infective endocarditis (3x). The vegetation near the apex remained visible on follow-up TTE. Diagnostic cardiac magnetic resonance imaging (CMR) was performed and showed left ventricular wall thickness, and diffuse subendocardial delayed enhancement in a patchy pattern. These findings are strongly suggestive of Loeffler's endocarditis. Bone marrow biopsy showed increased percentage of eosinophilic granulocytes, confirming the diagnosis of hypereosinophilic syndrome (HES).
The presence of infective endocarditis and simultaneous Loeffler's endocarditis has been reported once before by Menz et al. in 1994 published in Hertz. This case emphasizes the role of CMR as a diagnostic modality for Loeffler's endocarditis. Our patient had no peripheral hypereosinophilia. Myocardial biopsy conveys an increased risk for thromboembolic events and the possibility of a false negative result. CMR however, raised the suspicion of Loeffler's endocarditis, a diagnosis that was subsequently confirmed by bone marrow biopsy. The patient was treated with acenocoumarol and oral prednisone, which was gradually tapered after addition of mycophenolate mofetil.
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^∗: Present address: The New York Hospital-Cornell Medical Center, 525 East 68th Street, New York, New York 10021.

¹: From the Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases and the Cardiology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland.

^†: Present address: University of California, Irvine Medical Center, 101 City Drive South, Orange, California 92666.

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Clinical studyThe cardiovascular manifestations of the hypereosinophilic syndrome: Prospective study of 26 patients, with review of the literature

Abstract

Am J Med

Am J Med

Am J Med

The hypereosinophilic syndromes

Ann Intern Med

Eosinophilic leukemia. Report of five cases and review of the literature

Ann Intern Med

Eosinophilic leukemia, disseminated eosinophilic collagen vascular disease—a distinct entity

Acta Med Scand

The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature

Medicine (Baltimore)

Therapy of the hypereosinophilic syndrome

Ann Intern Med

Endocardiomyopathy with eosinophilia

Asymmetric septal hypertrophy (ASH): echocardiographic identification of the pathognomonic anatomic abnormality of IHSS

Circulation

Echocardiography

Echocardiography

Echocardiography

An improved method for echocardiographic detection of left atrial enlargement

Circulation

Relation between echocardiographically determined left atrial size and atrial fibrillation

Circulation

Clinical study
The cardiovascular manifestations of the hypereosinophilic syndrome: Prospective study of 26 patients, with review of the literature