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Progressive and restrictive cardiomyopathy associated
with a hypereosinophilic state and endocarditis resulting in impaired
ventricular filling with normal or decreased diastolic volume of either or
both ventricles, associated with multiple organ involvement.
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Löffler Endocarditis; Fibroplastic Parietal
Endocarditis with Eosinophilia; Nontropical Eosinophilic Endomyocardial
Fibrosis.
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Named after the Swiss Physician W. Löffler, who
described this disorder in 1936.
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Rare disorder that primarily occurs in people living in
the rain forest in the tropical and subtropical areas of Africa, Asia, and
South America. Males are affected more often than females.
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The condition has a predilection for African
populations, especially in tribes from Rwanda, which may or may not be
caused by genetic factors (yet unknown epidemiology of underlying
environmental factors may play the major role).
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Pathophysiology remains unclear, but many
hypotheses have been discussed. The basis of the cardiac injury seems to
involve eosinophil granule proteins that produce muscular and vascular
injury that in turn leads to endomyocardial fibrosis. How eosinophilic
infiltration and degranulation occur are unknown. Studies have shown that
interleukin-5 is produced by eosinophils and could have a role in chemotaxis
and degranulation.
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Based on clinical and biochemical findings such as a
high eosinophil blood count, but confirmation requires endocardial and/or myocardial
biopsy. The echocardiogram is not useful as a diagnostic tool in the early
stages of the disease. In the later stages, echocardiography may show a
markedly thickened endocardium and the chest radiograph a widespread
shadowing of both lung fields. Widespread T-wave inversions may be seen on
the electrocardiogram.
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Eosinophilic endomyocardial disease may be the
result of numerous disorders, including the idiopathic hypereosinophilic
syndrome, which mainly affects young men. Idiopathic hypereosinophilic
syndrome is a heterogeneous group of disorders characterized by an absolute
eosinophil count of more than 1500/mm3 that lasts for more than 6
months in the absence of any known cause of hypereosinophilia and with signs
of organ involvement. Cardiac involvement occurs in more than half of
patients and is the major cause of morbidity and mortality. However,
eosinophilic endomyocardial disease may also be associated with bronchial
carcinomas, medullary carcinoma of the thyroid, malignant fibrous
histiocytoma, eosinophilic leukemia, sarcoma, and several other disorders
(parasites, adverse drug reactions) associated with hypereosinophilia.
Patients with the disease initially may be asymptomatic or present with
tachypnea, shortness of breath, elevated central venous pressure, ascites,
and pulmonary edema as a consequence of global heart failure. The
endomyocarditis progresses in three different stages. The early necrotic
stage presents in up to one third of patients with systemic disease and
hypereosinophilia (unspecific signs such as fever, lymphadenopathy,
splenomegaly) and in up to half of the patients with signs of acute
endomyocarditis with inappetence, pulmonary infiltrates, cough, mitral and
tricuspid regurgitation, and global heart failure. Thromboembolic
complications may follow and can be found in up to 20% of patients. They
originate from mural thrombi over endomyocardial lesions and may lead to
anemic ...