Endocardial fibroelastosis

Endocardial fibroelastosis is a rare cardiac disorder characterized by diffuse thickening of the endocardium due to proliferation of fibrous and elastic tissue. This thickening can restrict ventricular filling and contraction, leading to heart failure, typically in infants and young children. It can be primary (idiopathic) or secondary to other cardiac anomalies.

This code is used when documentation specifies a diagnosis of endocardial fibroelastosis, whether primary or secondary. It is commonly seen in pediatric cardiology records for patients presenting with symptoms of heart failure, cardiomegaly, or arrhythmias. Supporting documentation includes echocardiogram findings, cardiac biopsy results, and physician notes confirming the diagnosis.

• Differentiate between primary and secondary forms if specified, although this code covers both.
• Code any associated heart failure (e.g., I50.-) or other congenital cardiac anomalies separately.
• Ensure the documentation clearly states "fibroelastosis" of the endocardium to avoid confusion with other forms of cardiomyopathy.