Endomyocardial fibrosis

This code represents a restrictive cardiomyopathy characterized by progressive fibrosis of the endocardium and subendocardium, primarily affecting the inflow tracts and apices of the ventricles. This scarring leads to impaired ventricular filling and can result in severe heart failure.

Use this code for documented cases of endomyocardial fibrosis, a rare heart muscle disease. Clinical documentation should specify the diagnosis of endomyocardial fibrosis, often supported by echocardiographic findings, cardiac MRI, or biopsy results demonstrating endocardial thickening and fibrosis.

• Differentiate from other restrictive cardiomyopathies, such as amyloidosis or hemochromatosis, which have distinct ICD-9-CM codes.
• Code any associated heart failure manifestations separately, such as congestive heart failure (428.0) or acute pulmonary edema (518.4).
• Ensure the medical record clearly supports the diagnosis of endomyocardial fibrosis, as it is a specific and uncommon condition.