What is a bicuspid aortic valve?
A normal aortic valve has three leaflets. A bicuspid aortic valve (BAV) is a condition in which the valve is born with two leaflets instead of three. The difference may seem minor, but it significantly changes how the valve functions.
Two leaflets must do the work of three — so they wear out faster, and earlier develop stenosis (narrowing) or regurgitation (backflow of blood). In some children this process happens quickly; in others there are no problems for decades.
BAV occurs in 1–2% of the population — making it one of the most common congenital heart defects. It is 2–3 times more frequent in boys than in girls.
"Children with a bicuspid aortic valve can look completely healthy for many years. This is precisely why, if a family member carries this diagnosis, children must be checked. Early detection makes treatment incomparably simpler."
Symptoms: what to watch for
Symptoms of BAV depend on the severity of the condition. In mild cases the child appears completely healthy — no symptoms, only a murmur heard on auscultation. In more severe cases:
- Easy fatigability with physical activity, shortness of breath
- Chest pain or pressure during exertion
- Heart murmur (the paediatrician may detect it during a check-up)
- Fainting or near-fainting — particularly during physical activity
- Dizziness after intensive exercise
- In infants: fatigue during feeding, slow weight gain
Important: BAV frequently causes no symptoms until complications develop. If a close relative (parent, sibling) has BAV — an echocardiogram for the child is recommended. This condition has a hereditary component.
Complications associated with BAV
A bicuspid valve is not always a problem on its own. The concern lies in the complications that can develop over time:
- Aortic stenosis — the valve narrows; the heart must work harder to pump blood. Over time, the left ventricle thickens and eventually fails to keep up.
- Aortic regurgitation — the valve fails to close fully; blood flows back into the ventricle. This too places extra load on the left ventricle.
- Aortic dilation — in patients with BAV, the aortic wall is structurally weaker. The aorta can enlarge; in rare cases the risk of dissection arises.
- Endocarditis — an altered valve is more vulnerable to bacterial infection.
These complications are what make regular monitoring essential — even children with BAV who have no symptoms need cardiology follow-up.
Diagnosis: how it is detected
In most cases BAV is first suspected when a paediatrician hears a systolic murmur during auscultation. The key investigation that confirms the diagnosis is echocardiography (echo) — it fully displays the valve's structure, its function, and the dimensions of the aorta.
Additional investigations may be needed: stress echo (to assess valve function under load), CT angiography (to clarify the condition of the aorta), MRI. Which investigations are required is determined by the clinical picture.
When is surgery needed?
The answer is not straightforward — every child is individual. But there are clear indications:
- Severe aortic stenosis — especially when symptoms are present (pain, fainting, shortness of breath)
- Significant deterioration in left ventricular size and function
- Aortic enlargement beyond a defined threshold
- Severe aortic regurgitation
Even without symptoms, the degree of severity on echo may necessitate intervention. This is exactly why dynamic monitoring is so important — we see changes before they become critical.
Treatment options
1. Balloon valvuloplasty (catheter-based)
If the stenosis is not severe and the child is young — the valve can be dilated with a balloon delivered by catheter, without opening the chest, through a vessel in the groin. This is a temporary solution, but sometimes allows the main operation to be deferred by several years.
2. Valve repair
Correcting the patient's own valve and preserving it — the preferred option. Not always possible, but when it is, this is what I choose: the patient's own tissue is always preferable to a prosthesis, especially for a growing child.
3. Valve replacement
If the valve can no longer be repaired — replacement with a prosthesis. Mechanical prostheses are very durable but require lifelong anticoagulation. Biological prostheses are less durable. Which is optimal for a specific child is determined by the clinical situation.
"Parents of children with BAV often gravitate to one of two extremes: 'it's nothing serious, let's wait' or 'we need to operate immediately.' The truth is in between — careful monitoring and intervention at exactly the right moment. Striking that balance is my job."
Life after surgery
With timely intervention, the outlook is good. Children go to school, participate in sport — however, patients with BAV require lifelong cardiology follow-up, since the aorta also needs to be monitored after surgery.
The question of sport is decided individually: with mild BAV most types of exercise are permitted; with severe stenosis or an enlarged aorta intensive exertion is restricted. An individualised plan agreed with the cardiologist is the best approach.
Questions and answers
Is BAV hereditary?
Yes. If a first-degree relative (parent, sibling) has BAV, the risk rises to 10–15%. For this reason, when the diagnosis is present in a family, echocardiographic screening of all close relatives is recommended.
My child has no symptoms — why is monitoring needed?
Because BAV can progress without symptoms — the aorta enlarges, valve function deteriorates. Regular echo shows these changes early, so that intervention can happen at the optimal moment.
Will the prosthesis last a lifetime?
A mechanical prosthesis is very durable but requires lifelong anticoagulants. A biological one lasts 10–20 years. When planning for a growing child we take into account that a further operation may be needed in the future — strategy is built with the long term in mind.
Can a child with BAV play sport?
It depends on the condition of the valve. With mild BAV most sports are permitted. With severe stenosis or an enlarged aorta, intensive exercise is restricted. The specific answer comes only after an individual cardiology consultation.
How often does an echo need to be done?
It depends on severity: with mild BAV — once a year; with more severe variants — more frequently. The specific intervals are set by the cardiologist.