Short answer · Medically reviewed summary · Last updated: 2026-05-08
The ICD-10 code for Bicuspid Aortic Valve is Q23.1 (congenital malformation of the aortic valve), while the legacy ICD-9 code is 746.4. These codes are primarily used for administrative and billing purposes to identify this specific congenital heart defect in medical records. What is Bicuspid Aortic Valve? Bicuspid Aortic Valve (BAV) is the most common congenital heart defect, affecting approximately 0.5% to 2% of the general population.
ICD10 code of Bicuspid Aortic Valve and ICD9 code
ICD-10 and ICD-9 codes for Bicuspid Aortic Valve, with classification details for clinicians, coders and patients.
The ICD-10 code for Bicuspid Aortic Valve is Q23.1 (congenital malformation of the aortic valve), while the legacy ICD-9 code is 746.4. These codes are primarily used for administrative and billing purposes to identify this specific congenital heart defect in medical records.
What is Bicuspid Aortic Valve?
Bicuspid Aortic Valve (BAV) is the most common congenital heart defect, affecting approximately 0.5% to 2% of the general population. In a healthy heart, the aortic valve has three leaflets (cusps) that open and close to regulate blood flow; in individuals with Bicuspid Aortic Valve, the valve has only two leaflets. This can lead to complications such as aortic stenosis, aortic regurgitation, or dilation of the ascending aorta over time.
Is Bicuspid Aortic Valve hereditary?
Research suggests a strong genetic component to Bicuspid Aortic Valve. While it is often sporadic, it frequently follows an autosomal dominant inheritance pattern with incomplete penetrance. Because family members of someone diagnosed with Bicuspid Aortic Valve may also have the condition without knowing it, clinical guidelines recommend screening first-degree relatives with an echocardiogram.
How is Bicuspid Aortic Valve managed?
Management of Bicuspid Aortic Valve focuses on monitoring for progression rather than immediate intervention. Our DiseaseMaps.org community of 142 members highlights that while many live asymptomatic lives, regular surveillance is vital. Common management strategies include:
- Regular transthoracic echocardiograms to monitor valve function and aortic diameter.
- Prophylactic blood pressure management to reduce stress on the aortic wall.
- Periodic cardiac MRI scans if the aorta requires more detailed imaging.
- Surgical intervention (valve repair or replacement) if the valve becomes severely stenotic or the aorta reaches a critical diameter.
Next steps
- Schedule a consultation with a cardiologist or a specialized congenital heart disease center to establish a monitoring baseline.
- Request genetic counseling if you are planning a family or have multiple affected relatives.
- Connect with the 142 members of the Bicuspid Aortic Valve community on DiseaseMaps.org to share experiences and coping strategies.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment from a qualified healthcare provider.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Bicuspid Aortic Valve.
- Orphanet: Congenital malformation of the aortic valve (ORPHA:99971).
- OMIM (Online Mendelian Inheritance in Man): Aortic Valve, Bicuspid (#109730).
- American Heart Association: Bicuspid Aortic Valve Disease Overview.
