Bicuspid aortic valve
Overview
Bicuspid aortic valve is a congenital heart defect characterized by an aortic valve with two cusps instead of the usual three. This valve, located between the left lower heart chamber and the aorta, regulates blood flow by opening and closing its cusps with each heartbeat. While most individuals have a tricuspid aortic valve, some may be born with a unicuspid valve (one cusp) or quadricuspid valve (four cusps), though these occurrences are rare. The proper functioning of the aortic valve is crucial to ensure blood flows in the correct direction throughout the body.
Changes to the aortic valve can lead to various health issues, which include:
- Aortic Valve Stenosis: This condition involves the narrowing of the aortic valve, hindering its full opening. As a result, blood flow from the heart to the body becomes reduced or blocked.
- Aortic Valve Regurgitation: In this situation, the aortic valve fails to close tightly, causing blood to flow backward.
- Aortopathy: This term refers to an enlarged aorta, which increases the risk of a tear in the aortic lining known as an aortic dissection.
Treatment for a bicuspid aortic valve depends on the severity of heart valve disease. It may include medicines, procedures and surgery.
Symptoms
Severe aortic stenosis or aortic regurgitation resulting from a bicuspid valve can lead to various symptoms. While most individuals with a bicuspid aortic valve may not experience symptoms of heart valve disease until adulthood, some infants may exhibit severe symptoms. Symptoms may include:
- Chest pain
- Shortness of breath
- Difficulty exercising
- Fainting or near fainting
Causes
The formation of a bicuspid aortic valve, the most common congenital heart defect present at birth, remains unknown to researchers. This condition develops early in pregnancy and may have a familial tendency, prompting individuals with affected family members to undergo heart tests. While it is not preventable, those diagnosed with a bicuspid aortic valve planning a pregnancy should consult their healthcare provider to closely monitor their condition and detect any potential issues early on.
Risk factor
Bicuspid aortic valves, which can be hereditary, require consultation with a doctor if a close family member has this heart defect. In such cases, medical professionals may conduct various tests to assess heart structure and function. If diagnosed with a bicuspid aortic valve and planning a pregnancy, seeking advice from a healthcare provider is essential. Although prevention is not possible, careful monitoring during pregnancy can help detect potential issues early on.
Diagnosis
A bicuspid aortic valve can be detected incidentally while conducting tests for other health concerns. A heart murmur may be heard during a routine examination. To confirm the diagnosis, an echocardiogram is used to create videos of the heart’s beating, showing blood flow through the heart chambers, valves, and the aorta. Additionally, a computed tomography (CT) scan is usually performed to check for any changes in the size of the aorta in individuals with a bicuspid aortic valve.
Treatment
Individuals with a bicuspid aortic valve are usually directed to a congenital cardiologist, specialized in congenital heart disease, for their healthcare needs. Regular checkups and imaging tests like echocardiograms are essential to assess the condition of the aortic valve for narrowing or leakage and to monitor any changes in the size of the aorta. Treatment approaches, which may involve medications, procedures, or surgery, depend on the severity of the heart valve disease.
Medications
While there are no medicines available to repair a bicuspid aortic valve itself, they can be utilized to manage symptoms caused by heart valve disease. For instance, blood pressure medicine might be recommended by your healthcare provider to address related issues.
Surgeries or other procedures
A bicuspid aortic valve may necessitate surgery if it is causing:
- Aortic valve stenosis.
- Aortic valve regurgitation.
- An enlarged aorta.
The aortic valve is surgically repaired or replaced. The precise heart valve issue and your symptoms will determine the sort of surgery that is performed.
- Aortic valve replacement. During the surgical procedure, the damaged valve is removed and substituted with either a mechanical valve or a biological tissue valve derived from cow, pig, or human heart tissue. In more complex cases, the aortic valve can be replaced with the patient’s own lung valve, which is then substituted with a lung tissue valve from a deceased donor, known as the Ross procedure. While biological tissue valves may degrade over time and require replacement, those with mechanical valves must take blood thinners indefinitely to prevent blood clots. The choice between valve types involves a thorough discussion between the patient and their healthcare provider, considering the associated benefits and risks of each option.
- Aortic root and ascending aorta surgery. During the procedure, surgeons remove the enlarged section of the aorta near the heart and replace it with a synthetic tube known as a graft, which is carefully sewn into position. In some cases, only the enlarged portion of the aorta is removed, leaving the aortic valve intact. Alternatively, the aortic valve can be either replaced or repaired during this surgical intervention.
- Balloon valvuloplasty. This heart valve procedure utilizes a thin and flexible tube known as a catheter with a balloon on its tip, making it an effective treatment for aortic valve stenosis in infants and children. However, in adults, the aortic valve often narrows again after the procedure, so it is typically reserved for those who are medically unfit to undergo surgery or are awaiting a valve replacement. During the process, the surgeon inserts the catheter into an artery in the arm or groin and guides it to the aortic valve. Once in position, the balloon on the catheter is inflated, enlarging the valve opening. Subsequently, the balloon is deflated, and both the catheter and balloon are removed.
