Endocardial fibroelastosis

Endocardial fibroelastosis (EFE) is a rare cardiac disorder characterized by a diffuse thickening of the endocardium (the inner lining of the heart chambers) due to proliferation of fibrous and elastic tissue. This thickening can impair ventricular function, leading to heart failure, arrhythmias, and sudden cardiac death, primarily affecting infants and young children.

This code is used for documented cases of endocardial fibroelastosis, typically diagnosed through echocardiography, cardiac MRI, or biopsy. It applies to both primary (idiopathic) and secondary forms of EFE, where the thickening is not attributable to other congenital heart defects or acquired conditions.

• Differentiate from other forms of cardiomyopathy or endomyocardial fibrosis, which have distinct ICD-9-CM codes.
• Ensure documentation specifies "fibroelastosis" to support this specific diagnosis.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter, especially in pediatric cardiology.