Accurate use of ICD-10-CM codes for rheumatic heart disease is essential for proper medical documentation and full payment in cardiology billing. Rheumatic heart disease (RHD) develops as a complication of acute rheumatic fever (ARF) and can lead to permanent damage of the heart valves, particularly the mitral and aortic valves. Because the disease can appear in both acute inflammatory stages and long-term chronic valve damage, selecting the correct ICD-10 code requires a clear understanding of the condition and its classification within the coding system.
In ICD-10-CM, rheumatic conditions are divided into two major code ranges: acute rheumatic fever codes (I00–I02) and chronic rheumatic heart disease codes (I05–I09).
The acute codes are used when there is active rheumatic inflammation affecting the heart, joints, or nervous system, while the chronic codes apply when rheumatic fever has resulted in long-term structural damage to the heart valves or other cardiac tissues.
Incorrectly coding for these categories is one of the most common mistakes in cardiology billing.
This guide provides a complete breakdown of ICD-10 codes for rheumatic heart disease, including codes for acute rheumatic fever, rheumatic chorea, and chronic rheumatic valve diseases affecting the mitral, aortic, and tricuspid valves.
ICD-10-CM Codes for Acute Rheumatic Fever (I00–I02)
These ICD-10 codes apply only when there is active, acute rheumatic inflammation. Medical billers should not use these codes for patients with chronic rheumatic valve disease who are not currently experiencing an acute episode.
The ICD codes for ARF are further divided in different categories i.e.:
ICD 10 Codes for Rheumatic Fever Without Heart Involvement (I00)
This ICD-10 code is used when rheumatic fever affects the body but no cardiac inflammation or damage is documented.
| ICD-10-CM Code | Diagnosis Description |
| I00 | Rheumatic fever without heart involvement |
ICD 10 Codes for Rheumatic Fever With Heart Involvement
These ICD 10 codes indicate active inflammation of the heart structures during acute rheumatic fever.
| ICD-10-CM Code | Diagnosis Description |
| I01 | Rheumatic fever with heart involvement (category) |
| I01.0 | Acute rheumatic pericarditis |
| I01.1 | Acute rheumatic endocarditis |
| I01.2 | Acute rheumatic myocarditis |
| I01.8 | Other acute rheumatic heart disease |
Rheumatic Chorea ICD 10 Codes
It is also known as Sydenham’s Chorea, this neurological manifestation of rheumatic fever is characterized by involuntary movements and behavioral changes. Specific ICD 10 codes are used i.e.,
| ICD-10-CM Code | Diagnosis Description |
| I02 | Rheumatic chorea (Sydenham’s chorea) |
| I02.0 | Rheumatic chorea with heart involvement |
| I02.9 | Rheumatic chorea without heart involvement |
ICD-10-CM Codes for Chronic Rheumatic Heart Disease (I05–I09)
Once the acute phase leads to permanent valve damage, the chronic rheumatic heart disease codes (I05–I09) apply. These codes are used even if the original rheumatic fever episode happened years or decades ago, as long as the valve condition is documented as rheumatic in origin.
ICD 10 Codes for Rheumatic Mitral Valve Diseases
These ICD-10 codes ranging from I05 to I05.9 cover chronic rheumatic damage affecting the mitral valve. The mitral valve is the most commonly affected valve in rheumatic heart disease.
| ICD-10-CM Code | Diagnosis Description |
| I05 | Rheumatic mitral valve diseases (category, non-billable) |
| I05.0 | Rheumatic mitral stenosis |
| I05.1 | Rheumatic mitral insufficiency (mitral regurgitation) |
| I05.2 | Rheumatic mitral stenosis with insufficiency |
| I05.8 | Other rheumatic mitral valve diseases |
| I05.9 | Rheumatic mitral valve disease, unspecified |
ICD 10 Codes for Rheumatic Aortic Valve Diseases (I06)
These ICD 10 codes represent rheumatic damage to the aortic valve. The aortic valve is the second most commonly affected valve in rheumatic heart disease.
| ICD-10-CM Code | Diagnosis Description |
| I06 | Rheumatic aortic valve diseases (category) |
| I06.0 | Rheumatic aortic stenosis |
| I06.1 | Rheumatic aortic insufficiency |
| I06.2 | Rheumatic aortic stenosis with insufficiency |
| I06.8 | Other rheumatic aortic valve diseases |
| I06.9 | Rheumatic aortic valve disease, unspecified |
ICD 10 Codes for Rheumatic Tricuspid Valve Diseases (I07)
These codes apply to rheumatic involvement of the tricuspid valve. Isolated tricuspid disease is rare and usually occurs alongside mitral disease.
| ICD-10-CM Code | Diagnosis Description |
| I07 | Rheumatic tricuspid valve diseases (category) |
| I07.0 | Rheumatic tricuspid stenosis |
| I07.1 | Rheumatic tricuspid insufficiency |
| I07.2 | Rheumatic tricuspid stenosis with insufficiency |
| I07.8 | Other rheumatic tricuspid valve diseases |
| I07.9 | Rheumatic tricuspid valve disease, unspecified |
ICD 10 Codes for Multiple Rheumatic Valve Diseases (I08)
Medical billers use these codes when more than one heart valve is affected by rheumatic disease. Coders should default to I08 codes rather than coding each valve separately when multi-valve involvement is documented.
| ICD-10-CM Code | Diagnosis Description |
| I08 | Multiple valve diseases (category) |
| I08.0 | Rheumatic disorders of both mitral and aortic valves |
| I08.1 | Rheumatic disorders of both mitral and tricuspid valves |
| I08.2 | Rheumatic disorders of aortic and tricuspid valves |
| I08.3 | Combined rheumatic disorders of mitral, aortic and tricuspid valves |
| I08.8 | Other multiple rheumatic valve diseases |
| I08.9 | Multiple rheumatic valve diseases, unspecified |
ICD 10 Codes for Other Rheumatic Heart Diseases (I09)
These codes include other or unspecified chronic rheumatic cardiac conditions not classified under the specific valve categories.
| ICD-10-CM Code | Diagnosis Description |
| I09.0 | Rheumatic myocarditis |
| I09.1 | Rheumatic diseases of endocardium, valve unspecified |
| I09.2 | Chronic rheumatic pericarditis |
| I09.81 | Rheumatic heart failure |
| I09.89 | Other specified rheumatic heart diseases |
| I09.9 | Rheumatic heart disease, unspecified |
Key Coding Tips for Rheumatic Heart Disease
Follow some easy coding tips to master entering ICD 10 codes properly for Rheumatic Heart Disease and get rheumatology billing done right.
Acute Vs. Chronic Matters
Use I00–I02 for the acute phase, and I05–I09 only for documented chronic structural damage.
Mitral Stenosis Vs. Mitral Regurgitation Default Coding
Per ICD-10-CM conventions, mitral stenosis without a documented cause is presumed rheumatic and coded to I05.0. However, mitral regurgitation without documentation of a rheumatic etiology should be coded as non-rheumatic mitral insufficiency (I34.0) unless the provider explicitly documents a rheumatic cause.
Use I08 For Multi-Valve Disease
When the documentation confirms more than one valve is affected, always use the combined I08 codes rather than separate I05, I06, and I07 codes.
Never Bill Non-Billable Category Codes
Parent codes such as I05, I06, I07, I08, and I09 are non-billable. Always select the most specific subcategory code available to avoid claim rejections.
Note: Always verify codes against the official ICD-10-CM tabular list before submission. Codes are valid for FY 2026 (October 1, 2025 – September 30, 2026).
