Clinically, obliterative cardiomyopathy is characterized by inexorably progressive congestive heart failure, systemic embolism, and cardiac arrhythmias and conduction disturbances. Although there may be a transient response to adrenal glucocorticoids or antitumor therapy, there is no effective treatment of this disorder. Worldwide, the most common cause of obliterative cardiomyopathy is endomyocardial fibrosis, which is particularly common in eastern Africa. Some researchers believe that many cases of endomyocardial fibrosis represent the end stage of a hypereosinophilic syndrome. Characteristically, there is massive endocardial thickening of both ventricles, although one ventricle may be more severely affected than the other.
Fibrous bands extend into the myocardium, and mitral and tricuspid insufficiency are common.
Patients usually die of congestive heart failure. Biventricular fibrotic involvement and the presence of mitral or tricuspid regurgitation are associated with poor long-term survival. There is no effective medical treatment. However, surgery consisting of endocardial stripping and relief of mitral or tricuspid insufficiency has been undertaken. The results have been reasonable, although the surgical mortality has been high.