Below is a brief summary of the common aortic and mitral valve disorders.

Aortic Valve Regurgitation
Aortic valve regurgitation (424.1) occurs when the aortic valve doesn’t close tightly and allows a backward flow of blood. Signs and symptoms include fatigue, shortness of breath, chest pain, fainting, rapid/irregular pulse, and heart palpitations. Some common causes of aortic valve regurgitation include rheumatic fever, congenital heart defect, deterioration of valve with age, endocarditis, syphilis, and ankylosing spondylitis. Aortic valve regurgitation, which most often develops gradually, is also called aortic insufficiency or aortic incompetence.

If the aortic regurgitation is specified as rheumatic, assign code 395.1 instead of code 424.1. Aortic regurgitation with aortic stenosis is classified to code 424.1 unless it is specified as rheumatic in which case code 395.2 is assigned. Aortic regurgitation with mitral valve involvement will be classified to code 396.1 or 396.3, depending on the type of mitral valve disorder.

Aortic Valve Stenosis
Aortic valve stenosis (424.1) occurs when the aortic valve narrows and prevents the valve from opening fully, which obstructs blood flow from the heart into the aorta. It causes pressure to build in the left ventricle and may lead to congestive heart failure (CHF).

Signs and symptoms include chest pain, chest tightness, feeling faint, dizziness, fatigue, shortness of breath, heart palpitations, and heart murmur. Some common causes of aortic stenosis include age, congenital heart defects, rheumatic fever, and calcium buildup on valve.

If the aortic stenosis is specified as rheumatic, assign code 395.0 instead. Aortic stenosis with aortic regurgitation/insufficiency/incompetence is classified to code 424.1 unless it is specified as rheumatic, in which case code 395.2 would be assigned. Aortic stenosis with mitral valve involvement will be classified to code 396.0 or 396.2, depending on the type of mitral valve disorder.

Mitral Valve Prolapse
Mitral valve prolapse (424.0) keeps the valve from closing properly. The leaflets of the mitral valve weaken, causing the blood to leak from the ventricle back into the atrium and may lead to mitral regurgitation. Common signs and symptoms of mitral valve prolapse include arrhythmia, dizziness, lightheadedness, shortness of breath, fatigue, cough, anxiety, and chest pain. Mitral valve prolapse may be caused by an inherited (genetic) condition, Marfan’s syndrome, lupus, rheumatic heart disease, endocarditis, coronary artery disease, and hyperthyroidism. Other names for mitral valve prolapse include the following:

• click-murmur syndrome;

• Barlow’s or Reid-Barlow’s syndrome;

• floppy valve syndrome;

• ballooning mitral valve syndrome or ballooning posterior leaflet syndrome;

• prolapsing mitral leaflet syndrome;

• myoxmatous mitral valve syndrome; and

• midsystolic-click-late systolic murmur syndrome.

Mitral Valve Regurgitation
Mitral valve regurgitation (424.0) occurs when the mitral valve does not close tightly, which allows blood to flow backward in the heart. Signs and symptoms include fatigue, shortness of breath, cough, heart palpitations, swollen feet or ankles, and heart murmur. Some common causes of mitral valve regurgitation include mitral valve prolapse, damaged cords that anchor the flaps of the mitral valve to the heart wall, rheumatic fever, endocarditis, deterioration of valve with age, prior heart attack, and congenital heart defects.

Mitral valve regurgitation is also called mitral insufficiency and mitral incompetence. If the mitral regurgitation is specified as rheumatic, assign code 394.1 instead. Mitral regurgitation with mitral stenosis is classified to code 394.2. Mitral regurgitation with aortic valve involvement will be classified to code 396.2 or 396.3, depending on the type of aortic valve disorder.

Mitral Valve Stenosis
Mitral valve stenosis (394.0) occurs when the mitral valve narrows, which causes the valve to not open properly and to obstruct blood flow between the left chambers of the heart. Blood cannot move efficiently through the heart. Common signs and symptoms include fatigue, shortness of breath, swollen feet and ankles, heart palpitations, dizziness, coughing, and respiratory infections. Mitral valve stenosis is most commonly caused by rheumatic fever and congenital heart defect.

AHA Coding Clinic for ICD-9-CM assumes that a diagnosis of mitral stenosis is rheumatic. If the patient also has mitral regurgitation/insufficiency/incompetence, then code 394.2 will be assigned instead. If the patient with mitral stenosis also has aortic valve involvement, then code 396.0 or 396.1 will be assigned depending on the type of aortic valve disorder. If the mitral stenosis is documented as not rheumatic, assign code 424.0.

Heart Valve Disorders With Congestive Heart Failure
Congestive heart failure (CHF) in a patient with mitral and aortic valve disease is presumed to be rheumatic and should be classified to 398.91, Rheumatic heart failure (congestive), unless the physician specifies a different cause. Therefore, code 428.0 cannot be assigned with a rheumatic heart disease code (394.x or 396.x) unless the physician specifies a different cause of the CHF (AHA Coding Clinic for ICD-9-CM, 1995, first quarter, page 6).

For example, a patient is admitted with mitral and aortic valve insufficiency and CHF. Mitral and aortic insufficiency is assigned to code 396.3, which is considered rheumatic in origin. Therefore, the CHF is also presumed to be rheumatic and code 398.91 is assigned for the rheumatic CHF.

Do not make the assumption that the CHF is rheumatic in nature unless AHA Coding Clinic for ICD-9-CM directs the coder to the rheumatic CHF code or the physician states the condition is rheumatic (AHA Coding Clinic for ICD-9-CM, 2000, second quarter, page 17).

Coding Heart Valve Disorders as a Secondary Diagnosis
To be a reportable secondary diagnosis, a condition must be documented by a physician in the body of the record and the condition must affect patient care in terms of requiring one of the following:

• clinical evaluation;

• therapeutic treatment;

• diagnostic procedures;

• extended length of hospitalization; or

• increased nursing care and/or monitoring.

Based on this information, it would be appropriate to add a code for a heart valve disorder that is documented in the medical record and evaluated, monitored, or treated during the current hospital stay. The evaluation, monitoring, or treatment may consist minimally of prophylactic antibiotics prior to surgery or a procedure, an ECHO, or transesophageal echocardiogram (TEE).

For example, a patient is admitted for diabetic neuropathy. During the admission, a TEE is performed to evaluate the St. Jude aortic valve. The TEE identifies mild to moderate mitral regurgitation, and the physician documents mild mitral regurgitation in the progress notes. The patient did not receive any medication changes because of the mitral regurgitation.

However, because the mitral regurgitation was evaluated by the TEE and the physician documented the condition in the progress notes, it would be appropriate to assign code 424.0 as a secondary diagnosis. If the physician documents the heart valve disorder in the past medical history section of the history and physical but the patient did not receive evaluation, monitoring, or treatment, then it would not be appropriate to assign a code for the condition unless the physician indicates the clinical significance of the condition.

Coding and sequencing for heart valve conditions are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding.

— This information was prepared by Audrey Howard, RHIA, of 3M Health Information Systems (800-367-2447), a supplier of coding and classification systems to nearly 5,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payors as the result of the misuse of this coding information.

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