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.
