Find information on thousands of medical conditions and prescription drugs.

Endomyocardial fibrosis

The hypereosinophilic syndrome is a disease process characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm3) in the blood for at least six months without any recognizable cause after a careful workup, with evidence of involvement of either the heart, nervous system, or bone marrow. more...

Home
Diseases
A
B
C
D
E
Ebola hemorrhagic fever
Ebstein's anomaly
Eclampsia
Ectodermal Dysplasia
Ectopic pregnancy
Ectrodactyly
Edwards syndrome
Ehlers-Danlos syndrome
Ehrlichiosis
Eisoptrophobia
Elective mutism
Electrophobia
Elephantiasis
Ellis-Van Creveld syndrome
Emetophobia
Emphysema
Encephalitis
Encephalitis lethargica
Encephalocele
Encephalomyelitis
Encephalomyelitis, Myalgic
Endocarditis
Endocarditis, infective
Endometriosis
Endomyocardial fibrosis
Enetophobia
Enterobiasis
Eosinophilia-myalgia...
Eosinophilic fasciitis
Eosophobia
Ependymoma
Epicondylitis
Epidermolysis bullosa
Epidermolytic hyperkeratosis
Epididymitis
Epilepsy
Epiphyseal stippling...
Epistaxiophobia
EPP (erythropoietic...
Epstein barr virus...
Equinophobia
Ergophobia
Erysipelas
Erythema multiforme
Erythermalgia
Erythroblastopenia
Erythromelalgia
Erythroplakia
Erythropoietic...
Esophageal atresia
Esophageal varices
Esotropia
Essential hypertension
Essential thrombocythemia
Essential thrombocytopenia
Essential thrombocytosis
Euphobia
Evan's syndrome
Ewing's Sarcoma
Exencephaly
Exophthalmos
Exostoses
Exploding head syndrome
Hereditary Multiple...
Hereditary Multiple...
Hereditary Multiple...
Hereditary Multiple...
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

There are two forms of the hypereosinophilic syndrome: Endomyocardial fibrosis and Loeffler's endocarditis. Endomyocardial fibrosis (also known as Davies disease) is seen in Africa and South America, while Loeffler's endocarditis does not have any geographic predisposition.

In both forms of the hypereosinophilic syndrome, the eosinophilia causes infiltration of the myocardium of the heart, which leads to fibrotic thickening of portions of the heart. The portions of the heart most effected by this disease are the apex of the left and right ventricles, fibrotic infiltrations may involve the mitral or tricuspid valves. Because of the infiltrative nature of the disease process, the cavity of the ventricles of the heart diminish in size, causing an obliterative cardiomyopathy and restriction to the inflow of blood in to the chambers of the heart. Ventricular mural thromb may develop.

Chronic eosinophilic leukemia (CEL) is a myeloproliferative disease which shares many common characteristics with hypereosinophilic syndrome. Many cases of CEL have a characteristic gene rearrangement , FIP1L1/PDGFRA, caused by a sub-micoscopic deletion of ~800 thousand base pairs of DNA on chromosome 4. The FIP1L1/PDGFRA fusion gene causes consitutive activation of the platelet derived growth factor receptor - alpha (PDGFRA). FIP1L1/PDGFRA-positive patients respond well to treatment with the tyrosine kinase inhibitor drug, imatinib mesylate (Gleevec® or Glivec®).

Read more at Wikipedia.org


[List your site here Free!]


Cardiac sarcoidosis could mimic giant cell myocarditis in a patient presenting with ventricular arrhythmias
From CHEST, 10/1/05 by Bassel Ramadan

INTRODUCTION: Giant cell myocarditis (GCM) is a rare, rapidly fatal myocarditis with histologic features that have some similarities to cardiac sarcoidosis (CS). We report a case of cardiac saecoidosis which initially was diagnosed as GCM. Howevere after a carefull review of the histopathlogical feature of the endomyocardial biopsy, and the presence of pulmonary granulomas on the transbronchial biopsy the diagnosis confirmed as a cadiac sarcoiodosis.

CASE PRESENTATION: 38 Year-old African American male, with no known medical problems, was transferred from an outside hospital to our institution for further workup of his ventricular arrhythmias. He had a syncopal episode while walking to the TV in his living room. He lost consciousness for 5-6 minutes and woke up without confusion, but he was complaining of chest pain. On admission the patient was noted to have multiple arrhythmias including second degree AV Block, nonsustained ventricular tachycardia, and torsades. He was complaining of some chest discomfort, cough, dyspnea, and 10 lbs weight loss. His physical exam was remarkable for elevated JVD; third heart sound, systolic murmur over the apex, bibasilar end inspiratory crackles, and 2 + pitting edema over the lower extremities. The electrolytes were within normal limits. Urine drug screen was negative and his TSH as well. Arterial blood gas on room air showed moderate degree of hypoxemia. Chest Ct with PE protocol did not show pulmonary embolus, but it showed diffuse pulmonary nodules with hilar and mediastinal lymphadenopathy. He underwent a cardiac catheterization which showed normal coronaries and ejection fraction of 15%. He had an endomyocardial biopsy as well and initially showed Giant Cell Myocarditis. Subsequently he had a bronchoscopy with transbronchial biopsy showing non-caseating granulomas suggestive of sarcoidosis (see figure 1). In view of the lung biopsy results the pathologist reevaluated his endomyocardial biopsy (see figure 2)and due to presence of non-caseating granulomas on endomyocardial biopsy, he revised the diagnosis to cardiac sarcoidosis. Patient underwent a placement of a cardiac defibrilator for his arrhythmias. Prednisone of 60mg a day was given with a marked improvement of his heart failure symptoms.

[FIGURE OMITED]

DISCUSSIONS: Idiopathic giant-cell myocarditis IGCM is a frequently fatal type of myocarditis. The clinical course, unlike that of sarcoid myocarditis CS, is usually characterized by progressive congestive heart failure, frequently associated with refractory ventricular arrhythmia. Most patients die of congestive heart failure. On the bases histopathology CS and IGCM may have similar characteristic. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS. The differentiation between CS and IGCM is clinically important, because the natural history of the disease, survival, and the treatment are different. For example, CS has better prognosis than IGCM, the 5 years survival is 80% for CS versus 20% for IGCM.The treatment for CS is corticosteroids. However, the treatment for IGCM is the combination of corticosteroids and cyclosporine which showed a survivor benefit compared with corticosteroids only.

CONCLUSION: The clinical cardiac involvement occurs in fewer than 5% of patients with sarcoidosis.CS and IGCM can look similar on the histopathology. Lung biopsy may help to Cardiac sarcoid patients treated with had 5-year survival rates 59-89%. However, the combination of corticosteroids and cyclosporine for patients with Giant Cell Myocarditis had 5-year survival rates of 10-18%.

REFERENCES:

(1) Cooper et al. New England Journal of Medicine 1997;336(26):1860-1866.

(2) Okura et al. American College of Cardiology. 41(2):322-9, 2003 Jan 15.

DISCLOSURE: Bassel Ramadan, None.

Bassel Ramadan MD * Emory University, Atlanta, GA

COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group

Return to Endomyocardial fibrosis
Home Contact Resources Exchange Links ebay