Congenital aortic valve stenosis involves a narrow opening in the aortic valve, restricting blood flow from the left ventricle to the aorta.
The aortic valve allows blood to flow from the left ventricle into the aorta, and prevents blood from flowing back into the left ventricle. It is normally made up of three leaflets, which open into three pockets called the aortic sinuses of Valsalva.
Patients can have an abnormal number of leaflets in their aortic valve, most commonly two leaflets (bicuspid aortic valve), and less commonly one (unicuspid) or four (quadricuspid). A bicuspid aortic valve often results in aortic valve stenosis.
Presentation
The symptoms depend on the severity of the narrowing. Mild aortic stenosis may be asymptomatic. Severe aortic stenosis presents with heart failure within months of birth.
Symptoms include:
- Fatigue
- Shortness of breath
- Dizziness
- Fainting
Symptoms are typically worse on exertion, as the outflow from the left ventricle cannot keep up with demand.
Signs
Aortic stenosis causes an ejection systolic murmur heard loudest in the aortic area, in the second intercostal space, right sternal border. It has a crescendo-decrescendo character and radiates to the carotids.
Other signs that may be present on examination are:
- Ejection click just before the murmur
- Palpable thrill during systole
- Slow-rising pulse and narrow pulse pressure
Management
An echocardiogram confirms the diagnosis.
Congenital aortic stenosis tends to worsen over time.
Treatment options include:
- Percutaneous balloon aortic valvuloplasty (inflating a balloon to stretch the valve via a catheter)
- Surgical aortic valvotomy (open surgery via a midline sternotomy incision to widen the aortic valve)
- Valve replacement (open surgery via a midline sternotomy incision to replace the aortic valve)
Last updated January 2025
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