2020 ICD-10-CM Diagnosis Code I42.4

Endocardial fibroelastosis

    2016 2017 2018 2019 2020 Billable/Specific Code
  • I42.4 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM I42.4 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of I42.4 - other international versions of ICD-10 I42.4 may differ.
Applicable To
  • Congenital cardiomyopathy
  • Elastomyofibrosis
The following code(s) above I42.4 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to I42.4:
  • I00-I99
    2020 ICD-10-CM Range I00-I99

    Diseases of the circulatory system

    Type 2 Excludes
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    • systemic connective tissue disorders (M30-M36)
    • transient cerebral ischemic attacks and related syndromes (G45.-)
    Diseases of the circulatory system
  • I42
    ICD-10-CM Diagnosis Code I42

    Cardiomyopathy

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Code First
    • pre-existing cardiomyopathy complicating pregnancy and puerperium (O99.4)
    Includes
    • myocardiopathy
    Type 2 Excludes
    • ischemic cardiomyopathy (I25.5)
    • peripartum cardiomyopathy (O90.3)
    • ventricular hypertrophy (I51.7)
    Cardiomyopathy
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.
ICD-10-CM I42.4 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 314 Other circulatory system diagnoses with mcc
  • 315 Other circulatory system diagnoses with cc
  • 316 Other circulatory system diagnoses without cc/mcc

Convert I42.4 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to I42.4:
  • Type 1 Excludes: I38
    , Q24
    ICD-10-CM Diagnosis Code I38

    Endocarditis, valve unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Includes
    • endocarditis (chronic) NOS
    • valvular incompetence NOS
    • valvular insufficiency NOS
    • valvular regurgitation NOS
    • valvular stenosis NOS
    • valvulitis (chronic) NOS
    Type 1 Excludes
    • congenital insufficiency of cardiac valve NOS (Q24.8)
    • congenital stenosis of cardiac valve NOS (Q24.8)
    • endocardial fibroelastosis (I42.4)
    • endocarditis specified as rheumatic (I09.1)
    ICD-10-CM Diagnosis Code Q24

    Other congenital malformations of heart

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • endocardial fibroelastosis (I42.4)

Diagnosis Index entries containing back-references to I42.4:
  • Anomaly, anomalous (congenital) (unspecified type) Q89.9
    ICD-10-CM Diagnosis Code Q89.9

    Congenital malformation, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital anomaly NOS
    • Congenital deformity NOS
    • heart Q24.9
      ICD-10-CM Diagnosis Code Q24.9

      Congenital malformation of heart, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
      Applicable To
      • Congenital anomaly of heart
      • Congenital disease of heart
      • fibroelastosis cordis I42.4
  • Cardiomyopathy (familial) (idiopathic) I42.9
    ICD-10-CM Diagnosis Code I42.9

    Cardiomyopathy, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Cardiomyopathy (primary) (secondary) NOS
    • newborn I42.8
      ICD-10-CM Diagnosis Code I42.8

      Other cardiomyopathies

        2016 2017 2018 2019 2020 Billable/Specific Code
      • congenital I42.4
    • congenital I42.4
  • Elastomyofibrosis I42.4
  • Fibroelastosis I42.4 (cordis) (endocardial) (endomyocardial)
  • Newborn (infant) (liveborn) (singleton) Z38.2
    ICD-10-CM Diagnosis Code Z38.2

    Single liveborn infant, unspecified as to place of birth

      2016 2017 2018 2019 2020 Billable/Specific Code Newborn/Neonate Dx (0 years) POA Exempt
    Applicable To
    • Single liveborn infant NOS
  • Sclerosis, sclerotic
    • subendocardial, congenital I42.4

ICD-10-CM Codes Adjacent To I42.4
I40.0 Infective myocarditis
I40.1 Isolated myocarditis
I40.8 Other acute myocarditis
I40.9 Acute myocarditis, unspecified
I41 Myocarditis in diseases classified elsewhere
I42 Cardiomyopathy
I42.0 Dilated cardiomyopathy
I42.1 Obstructive hypertrophic cardiomyopathy
I42.2 Other hypertrophic cardiomyopathy
I42.3 Endomyocardial (eosinophilic) disease
I42.4 Endocardial fibroelastosis
I42.5 Other restrictive cardiomyopathy
I42.6 Alcoholic cardiomyopathy
I42.7 Cardiomyopathy due to drug and external agent
I42.8 Other cardiomyopathies
I42.9 Cardiomyopathy, unspecified
I43 Cardiomyopathy in diseases classified elsewhere
I44 Atrioventricular and left bundle-branch block
I44.0 Atrioventricular block, first degree
I44.1 Atrioventricular block, second degree
I44.2 Atrioventricular block, complete

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.