2027 ICD-10-CM Diagnosis Code I42.01Familial-genetic dilated cardiomyopathy
ICD-10-CM Codes›I00–I99›I30-I5A›I42
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- New 2027 Code
I42.01 is a billable ICD-10-CM diagnosis code for familial-genetic dilated cardiomyopathy. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 314 through 316. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 117 closely related codes.
Code Identity
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Autosomal dominant epidermolysis bullosa simplex
- Autosomal recessive familial wooly hair
- Cardiomyopathy with cataract and hip spine disease syndrome
- Congenital wooly hair
- Dilated cardiomyopathy 3B
- Dilated cardiomyopathy with genetic marker
- Dilated cardiomyopathy with hypergonadotropic hypogonadism syndrome
- Erythrokeratodermia cardiomyopathy syndrome
- Familial cardiomyopathy
- Familial dilated cardiomyopathy with conduction defect due to LMNA mutation
- Intermediate epidermolysis bullosa simplex with cardiomyopathy
- Microcephalus cardiomyopathy syndrome
- Nonsenile cataract
- Primary dilated cardiomyopathy
- Primary familial dilated cardiomyopathy
- Sensorineural deafness with dilated cardiomyopathy syndrome
- Wooly hair and palmoplantar keratoderma with dilated cardiomyopathy syndrome
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to I42.01: its own notes plus those printed at I42 and Chapter 9. A note printed at a category, block or chapter applies to every code under it.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- 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.-)
Code First
The underlying condition named in the note is sequenced before this code.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name I42.01, its category, or a range that includes it.
Applicable To 1
These notes mention I42.01.
Code First 1
These codes say to code first a condition named in the note, and I42.01 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical InformationClinical
Cardiomyopathy, Dilated
a form of cardiac muscle disease that is characterized by ventricular dilation, ventricular dysfunction, and heart failure. risk factors include smoking; alcohol drinking; hypertension; infection; pregnancy; and mutations in the lmna gene encoding lamin type a, a nuclear lamina protein.
Code History & ChangesHistory
New Code I42.01 was added to the ICD-10-CM code set for FY 2027, effective October 1, 2026.
Replacement I42.01 replaces the following previously assigned code(s):
- I42.0 - Dilated cardiomyopathy
Questions About I42.01Overview
What is the ICD-10 code for familial-genetic dilated cardiomyopathy?
The ICD-10-CM code for familial-genetic dilated cardiomyopathy is I42.01 (sometimes written as I4201). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is I42.01 (Dilated cardiomyopathy) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report familial-genetic dilated cardiomyopathy on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does I42.01 group to?
When familial-genetic dilated cardiomyopathy is the principal diagnosis on an inpatient stay, it groups to MS-DRG 314, 316, 315, with relative weights from 0.6678 to 2.0871 depending on complications. Higher weights mean higher Medicare reimbursement.
Is I42.01 a CC or MCC?
CMS lists I42.01 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 117 closely related codes in its exclusion list.
