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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.

Löffler Endocarditis; Fibroplastic Parietal Endocarditis with Eosinophilia; Nontropical Eosinophilic Endomyocardial Fibrosis.

Named after the Swiss Physician W. Löffler, who described this disorder in 1936.

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.

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).

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.

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.

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 ...

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