Endomyocardial fibrosis is a disorder of uncertain etiology that can affect the left, right or both ventricles. The number of cases have been declining for unknown reasons.


Pathology: subendocardial fibrosis of the right ventricle


Pathologic changes:

(1) restrictive cardiomyopathy

(2) tricuspid valve regurgitation (due to tethering of the papillary muscles)

(3) right atrial enlargement


Clinical features:

(1) diastolic dysfunction of the right ventricle

(2) ascites, often severe

(3) little or no ankle edema

(4) high venous pressure with dominant systolic wave (secondary to tricuspid regurgitation)

(5) loud third heart sound

(6) variable pericardial effusion

(7) variable atrial fibrillation, usually without a fast ventricular response


The diagnosis can be made by echocardiography or cardiac imaging method, which demonstrates the subendocardial fibrosis and tricuspid valve changes.


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