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Published on: May 10, 2024
Hypereosinophilic syndrome: cardiac diagnosis and management
Rekha Mankad1, Crystal Bonnichsen1, Sunil Mankad1
1Division of Cardiology, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Hypereosinophilic syndrome (HES) involves high eosinophil counts causing organ damage, particularly cardiac dysfunction. Early diagnosis and targeted treatments are crucial for managing this rare condition.
Area of Science:
- Hematology
- Cardiology
- Immunology
Background:
- Hypereosinophilic syndrome (HES) is characterized by persistent hypereosinophilia (HE) and associated organ damage.
- Cardiac dysfunction is a frequent and severe complication of HES, contributing significantly to morbidity and mortality.
Purpose of the Study:
- To outline the diagnostic approach for HES, emphasizing confirmation of hypereosinophilia and investigation of organ involvement.
- To detail the cardiac manifestations of HES and the diagnostic tools used for assessment.
- To present the current treatment strategies for HES, including medical management and potential interventions.
Main Methods:
- Confirmation of hypereosinophilia through repeat blood testing.
- Comprehensive etiological investigation to identify the cause of HE.
- Cardiac assessment using echocardiography, cardiac MRI, CT, and endomyocardial biopsy.
- Treatment guided by etiology, including corticosteroids, imatinib, anticoagulation, heart failure therapy, and transplantation.
Main Results:
- Cardiac dysfunction, including apical obliteration, thrombi, and restrictive cardiomyopathy, is a common finding in HES.
- Endomyocardial biopsy is the gold standard for diagnosing cardiac involvement.
- Treatment strategies aim to reduce eosinophil counts and manage organ damage.
Conclusions:
- Prompt diagnosis and management of HES are essential to prevent severe organ damage, especially cardiac complications.
- A multidisciplinary approach involving hematology, cardiology, and immunology is crucial for optimal patient outcomes.
- Treatment decisions for HES should be individualized based on the underlying cause and extent of organ involvement.
Abstract:
Hypereosinophilic syndrome (HES) is a heterogeneous group of conditions that is defined at its core by hypereosinophilia (HE) (blood eosinophil count of >1.5×10(9)/L) and organ damage directly attributable to the HE. Cardiac dysfunction occurs frequently in all forms of HES and is a major cause of morbidity and mortality. Once a significantly elevated eosinophil count is identified, it must be confirmed on repeat testing and the aetiology for the HE must be rigorously sought out with a focus on identifying whether organ dysfunction is occurring. Echocardiography is routinely performed to assess for cardiac involvement, looking for evidence of left ventricular and/or right ventricular apical obliteration or thrombi or a restrictive cardiomyopathy. Cardiac magnetic resonance imaging and CT are often useful adjuncts to establish the diagnosis but endomyocardial biopsy remains the gold standard. To decrease the degree of eosinophilia, treatment can include corticosteroids and/or imatinib based on the aetiology. Anticoagulation, standard heart failure therapy for a restrictive cardiomyopathy and finally cardiac transplantation may be indicated in the treatment algorithm.
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