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. Symptoms include severe peripheral edema and breathlessness. The disease is rapidly fatal.
Löffler Endocarditis; Fibroplastic Parietal Endocarditis with Eosinophilia; Nontropical Eosinophilic Endomyocardial Fibrosis.
Named after the Swiss Physician Willem 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).
Eosinophilic states causing Hypereosinophilic Syndrome such as eosinophilic leukemia, carcinoma, lymphoma, drug reactions or parasites, have been reported in the literature to result in Löffler Syndrome. Hypereosinophilia may result from a worm called “Helminth.” This worm causes a persistent increase in eosinophils that results in Hypereosinophilic Syndrome. The basis of the cardiac injury seems to involve eosinophil granule proteins that penetrate cardiac myocytes causing thickening of the muscle and subsequently vascular injury that in turn leads to endomyocardial fibrosis. How eosinophilic infiltration and degranulation occur remains unknown. Commonly the heart develops large mural thrombi as a result of the deterioration of left ventricular wall muscle. The disease is commonly contracted in temperate climates because of the favorable conditions for parasite infestations. It is rapidly fatal. 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. High eosinophil blood count and endocardial and/or myocardial biopsy are essential to establish the diagnosis. 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. Whereas earlier studies reported that up to ...