The aortic valve is the valve between the left ventricle and the aorta. Aortic valve stenosis (also referred to as aortic stenosis) occurs when the aortic valve is narrowed or thickened and causes obstruction. This makes it hard for the heart to pump blood to the body, and the left ventricle needs to squeeze at a higher pressure to overcome the obstruction.
Aortic valve stenosis (narrowing) or regurgitation (leaking) may affect all ages — infants, children, adolescents, and adults. A normal aortic valve has three leaflets or cusps, but a stenosed valve may have only one cusp (unicuspid) or two cusps (bicuspid).
Aortic stenosis can have different levels of severity such as mild, severe, and critical. When the level of stenosis is considered severe or critical, an intervention needs to be performed immediately to allow blood to go to the body. Some patients with aortic valve defects also have an enlarged aortic annulus. The annulus is a fibrous ring between the left ventricle and the aorta, where the aortic valve is seated. If the annulus is enlarged, the aortic valve cusps have difficulty meeting, and the valve may become regurgitant (leaky).
Aortic Valve Stenosis
What Are Symptoms of Aortic Valve Stenosis?
Children with mild or even moderate aortic stenosis usually do not have symptoms. If symptoms occur, they may include:
With severe/critical narrowing, there may be symptoms at birth which consist of:
Possible mottled and cool skin
How Is Aortic Valve Stenosis Diagnosed?
Aortic stenosis is diagnosed after a physician takes a history and performs a physical examination. An abnormal heart murmur is usually noted on physical examination along with other features, which suggest the diagnosis of aortic stenosis.
With severe or critical aortic stenosis, the abnormal murmur may not be present, though there are findings of shock which suggest the possibility of a heart defect. The diagnosis and severity of the aortic stenosis is confirmed with an echocardiogram. In certain circumstances aortic stenosis is detected prenatally during a fetal echocardiogram.
How Is Aortic Valve Stenosis Treated?
The need for intervention depends on how severe the stenosis is, size of the patient, the possibility of needing an artificial valve and the specific nature of the valve abnormality.
In very severe or critical aortic stenosis, an intervention is required soon after birth to try to achieve normal blood flow to the body. Initially, a drug called prostaglandin may be given to keep the ductus arteriosus open to allow blood flow from the pulmonary artery to the aorta. The prostaglandin treatment is only temporary since procedures are needed to relieve the obstruction and allow enough blood to get to the body.
Cardiac Catheterization Intervention
If the left ventricle is an adequate size, the obstruction across the aortic valve may be relieved by an interventional cardiac catheterization procedure. The procedure involves placing and then inflating a balloon across the valve. This dilates the valve narrowing and is called balloon valvuloplasty.
If the interventional catheterization is not possible or successful, open-heart surgeryis needed to relieve the obstruction. This can include aortic valve repair or aortic valve replacement.
Aortic valve replacement is recommended only when repair will likely not result in a functioning valve. Valve replacement in children is challenging as noted with the options listed below.
Types of aortic valve replacement include:
The Ross procedure – This replaces the patient's aortic valve with their own pulmonary valve, and the pulmonary valve is then replaced with a biologic valve. Therefore, the patient’s aortic valve can grow with the patient. However, the valve used to replace the pulmonary valve will need to be replaced in the future.
Mechanical Valves (having metal parts in the valve) – This is a long-lasting solution. However, mechanical valves do not grow with the child and may need replacement with a larger size valve. They require blood-thinning medication to prevent blood clots from forming on the metal valve. In an active toddler or child, avoiding bumps and falls can be challenging; in a teen who wishes to participate in recreational or competitive sports, injury is a hazard.
Biologic Valves (porcine or bovine - pig or cow) – These do not grow with the patient, but do not require taking blood thinners. They usually do not last as long as mechanical valves. They may result in valve calcifying (hardening) more quickly, requiring reoperations to replace calcified valves Children’s metabolism is different from adults and may result.
To help prepare families for their care with Lurie Children's Heart Center, we have compiled a list of resources about treatment and recovery. Learn how to get ready for an inpatient stay or outpatient visit, and read about our support services for patients and families.