Eosinophilic cardiomyopathy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Eosinophillic Cardiomyopathy also known as Loeffler's Syndrome is a type of restrictive cardiomyopathy caused by eosinophillic infiltration of the endomyocardium. It is a feature of hypereosinophilic syndrome and occurs in 40-50% of all cases. Eosinophills enter the tissue and undergo degranulation; release cytotoxic proteins; increase production of reactive oxygen species, enzymes, growth factor, and cytokines. This process leads to tissue damage and dysfunction, eventually leading to fibrosis and restrictive cardiomyopathy. There are 3 stages of the eosinophilic cardiomyopathy: Stage 1 that is acute necrotic stage, stage 2 that is mural thrombus formation, and stage 3 that is ibrotic stage. There are many causes of hypereosinophilic syndrome (HES) with eosinophilic cardiomyopathy, these can be classified as reactive, conal myeloid disorder, and idiopathic hypereosinophillic syndrome.

Historical Perspective

• Eosinophillic Cardiomypoathy was first discovered by Wilhem Loeffler, a Swiss Physician, in 1936.

Classification

• Eosinophilic Cardiomyopathy may be classified according to cause into three subtypes/groups:

• Reactive
• Clonal myeloid disorder
• Idiopathic hypereosinophillic syndrome

Pathophysiology

• The pathogenesis of eosinophilic cadiomyopathy is characterized by hypereosinophilia , thrombi formation , restrictive cardiomyopathy , and heart failure .
• The FLP1L1-PDGFRA fusion gene has been associated with the development chronic eosinophilic leukemia and hypereosiniophilic syndrome.
• The PDGFRa, PDGFRb, and PDGFR1 genes have been associated with the development of clonal myeloid disorders leading to hypereosiniophilic syndrome.
• On gross pathology, cardiac hypertrophy and fibrosis are characteristic findings of Eosinophilic Cardiomyopathy.
• On microscopic histopathological analysis, hypereosinophilia, myocardial and myofibrillar disarray, and endoperimysial interstitial fibrosis are characteristic findings of Eosinophilic Cardiomyopathy.

Clinical Features

• Dyspnea
• Chest pain
• Cough
• Embolic events
• Fever

Differentiating [disease name] from other Diseases

• Eosinophilic cardiomyopathy must be differentiated from other diseases that cause cardiac hypertrophy, cardiac fibrosis, and heart failure, such as:

• Amyloidosis
• Sarcoidosis
• Hemachromatosis

Epidemiology and Demographics

• The prevalence of hypereosinophilic syndrome is approximately 0.036 per 100,000 individuals worldwide.

Age

• Patients of all age groups may develop ecosinophilic cardiomyopathy.
• Eosinopilic cardiomyopathy is more commonly observed among patients aged 20-50 years old.

Gender

• Men are more commonly affected with eosinophilic cadiomyopathy than Females.
• The male to female ratio is approximately 1.47.

Race

• There is no racial predilection for eosinophilic cardiomyopathy.

Risk Factors

• Common risk factors in the development of eosinophilic cardiomyopathy are HLA Bw44 positivity, splenomegaly, thrombocytopenia, elevated vitamin B12 levels, dysplastic eosinophils, and abnormal early myeloid precursors.

Natural History, Complications and Prognosis

• Some patients with eosinophilic cardiomyopathy may present asymptomatic.
• Early clinical features include dyspnea, fever, and chest pain.
• If left untreated, patients with eosinophilic cardiomyopathy may progress to develop heart failure, arrythmias, and sudden cardiac death.

• Prognosis is generally good, and depends on its etiology.

Diagnosis

Diagnostic Criteria

• The diagnosis of hypereosinophilic syndrome is made when at least one of the following three diagnostic criteria are met:

• An elevated eosinophil count (>1500/mm3) on 2 separate tests (≥1 mo).
• Tissue hypereosinophilia based on >20% eosinophils in a bone marrow section.
• Marked disposition of eosinophilic granule proteins in tissue plus organ damage directly due to hypereosinophilia.

Symptoms

• Eosinophilic cardiomyopathy is may present asymptomatic early in the course.
• Symptoms of eosinophilc cardiomypoathy may include the following:
  • Fever
  • Dyspnea at rest or on exertion
  • Chest pain
  • Cough
  • Rash
  • Malaise

Physical Examination

• Patients with eosinophilic cardiomyopathy usually appear distressed.
• Physical examination may be remarkable for:
  • Fever
  • Tachycardia
  • Tachypnea
  • Arrythmia
  • Rash
  • Embolic events

Laboratory Findings

• A positive cardiac biopsy is diagnostic of eosinophilic cardiomyopathy.
• An elevated concentration of eosinophils in peripheral blood smear is diagnostic of hypereosinophilic syndrome.
• Other laboratory findings consistent with the diagnosis of eosinophilic cardiomyopathy include:
  • Abnormal bone marrow biopsy
  • Abnormal troponins

Imaging Findings

• Cardiac MRI is the imaging diagnostic study of choice for eosinophilic cardiomyopathy.
• On cardiac MRI, eosinophilic cardiomyopathy is characterized by hyper-enhancement and thrombus formation.
• Echocardiography may demonstrate:
  • Endomyocardial thickening
  • Ventricular thrombus formation
  • Mitral valve involvement

Other Diagnostic Studies

• Eosinophilic cardiomyopathy may also be diagnosed using ECG.
• Findings on ECG include:
  • T wave inversion
  • Left atrial enlargement
  • Left ventricular hypertrophy
  • Right bundle branch block

Treatment

Medical Therapy

• The treatment of eosinophilic cardiomyopathy depends on the underlying cause.
• The mainstay of therapy is supportive care.
• The mainstay of therapy for eosinophilic cardiomyopathy with a FIP1L1-PDGFRA mutation is imatinib and steroids.
• Symptomatic management includes:
  • Diuretics
  • Beta Blockers
  • ACE inhibitors
  • Angiotensin Receptor Blockers
  • Digoxin
• Imatinib acts by inhibition of tyrosine kinase.
• Response to treatment can be monitored with Echocardiography.

Surgery

• Valve replacement may be performed for patients with valvular damage in eosinophilic cardiomyopathy.
• Cardiac transplant may be performed for patients with irreversible damage in eosinophilic cardiomyopathy.

Prevention

• There are no primary preventive measures available for eosinophilic cardiomyopathy.

• Once diagnosed and successfully treated, patients with eosinophilic cardiomyopathy are followed-up. Follow-up testing includes Echocardiography and Electrocardiogram.