I42.4

Applicable To

• Congenital cardiomyopathy

• Elastomyofibrosis

Approximate Synonyms

• Arrhythmogenic right ventricular dysplasia

• Cardiomyopathy restrictive, endocardial fibroelastosis

• Congenital arrhythmogenic right ventricular dysplasia

• Congenital endocardial fibroelastosis at birth)

• Primary endocardial fibroelastosis

• Primary endocardial fibroelastosis heart condition)

• Restrictive cardiomyopathy secondary to endocardial fibroelastosis

Clinical Information

• A condition characterized by the thickening of endocardium due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function cardiomyopathy, restrictive). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies heart defects congenital;) infection; or gene mutation. Defects in the tafazzin protein, encoded by taz gene, result in a form of autosomal dominant familial endocardial fibroelastosis.

• A rare disorder characterized by diffuse thickening of the endocardium. It presents with unexplained heart failure.

• Condition characterized by hypertrophy of the wall of the left ventricle and conversion of the endocardium into a thick fibroelastic coat, with the capacity of the ventricle sometimes reduced, but often increased.

Details

• I42.4 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM I42.4 became effective on October 1, 2022.

• This is the American ICD-10-CM version of I42.4 – other international versions of ICD-10 I42.4 may differ.