2024 ICD-10-CM Diagnosis Code I42.4

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Cardiomyopathy and renal anomaly syndrome
• Congenital endocardial fibroelastosis
• Congenital endocardial fibroelastosis
• Endocardial fibroelastosis
• Endocardial fibroelastosis
• Endocardial fibroelastosis of left atrium
• Endocardial fibroelastosis of right atrium
• Hydrocephalus with endocardial fibroelastosis and cataract syndrome
• Primary endocardial fibroelastosis
• Primary endocardial fibroelastosis of left ventricle
• Primary endocardial fibroelastosis of right ventricle
• Restrictive cardiomyopathy secondary to endocardial fibroelastosis
• Secondary restrictive cardiomyopathy

Clinical Information

• Endocardial Fibroelastosis

  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.

• Endocardial Fibroelastosis

  a rare disorder characterized by diffuse thickening of the endocardium. it presents with unexplained heart failure.

• TAFAZZIN wt Allele|BTHS|Barth Syndrome Gene|CMD3A|Cardiomyopathy, Dilated 3A (X-Linked) Gene|EFE|EFE2|Endocardial Fibroelastosis 2 Gene|G4.5|LVNCX|TAZ|TAZ1|Tafazzin, Phospholipid-Lysophospholipid Transacylase wt Allele|Taz1

  human tafazzin wild-type allele is located in the vicinity of xq28 and is approximately 10 kb in length. this allele, which encodes tafazzin protein, plays a role in phospholipid metabolism, including cardiolipin remodeling. mutations in the gene are associated with barth syndrome, dilated cardiomyopathy (dcm), hypertrophic dcm, endocardial fibroelastosis and left ventricular noncompaction.

I42.4 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

MS-DRG	MS-DRG Title	MCD	Relative Weight
314	OTHER CIRCULATORY SYSTEM DIAGNOSES WITH MCC	05	2.0935
315	OTHER CIRCULATORY SYSTEM DIAGNOSES WITH CC	05	0.9673
316	OTHER CIRCULATORY SYSTEM DIAGNOSES WITHOUT CC/MCC	05	0.6927

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Convert I42.4 to ICD-9-CM

• ICD-9-CM Code: 425.3 - Endocard fibroelastosis

Patient Education

Cardiomyopathy

Cardiomyopathy is the name for diseases of the heart muscle. These diseases enlarge your heart muscle or make it thicker and more rigid than normal. In rare cases, scar tissue replaces the muscle tissue.

Some people live long, healthy lives with cardiomyopathy. Some people don't even realize they have it. In others, however, it can make the heart less able to pump blood through the body. This can cause serious complications, including:

• Heart failure
• Abnormal heart rhythms
• Heart valve problems
• Sudden cardiac arrest (SCA)

Heart attacks, high blood pressure, infections, and other diseases can all cause cardiomyopathy. Some types of cardiomyopathy run in families. In many people, however, the cause is unknown. Treatment might involve medicines, surgery, other medical procedures, and lifestyle changes.

NIH: National Heart, Lung, and Blood Institute

[Learn More in MedlinePlus]

Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.