Congenital Aortic Valve Stenosis

What is it?
Aortic valve stenosis is a narrowing of the aortic valve. The aortic valve opens to allow blood to flow from the left ventricle of the heart to the aorta, and then to the body. Because the opening is narrowed, the left ventricle has to pump extra hard to propel blood through the valve. This causes the left ventricle to become enlarged. The valve may also have two leaflets, instead of three, or may have an abnormal funnel shape. The narrowed valve may not be a problem during infancy, but becomes one as the child grows and the heart cannot keep up with the body’s demand for blood. “Congenital” aortic valve stenosis means the condition is present at birth.
Who gets it?
Males are four times more likely to get this disease. Aortic valve stenosis accounts for approximately 7% of all cases of congenital heart disease.
What causes it?
Aortic valve stenosis can run in families or may be due to something that happened to the fetus during pregnancy.
Unless it is severe, the condition is often not detected until later in life.
What are the symptoms?
Most children with aortic valve stenosis have no symptoms, so it’s difficult to detect. In general, many patients will be easily fatigued, but show no other symptoms until their thirties to fifties. A small number of children may be prone to dizziness and fainting (syncope) within the first ten years of life. If the obstruction is great, infants may suffer from severe heart failure. Sudden death is uncommon, but possible. Adolescents with hypertrophic cardiomyopathy, a type of aortic stenosis where the left ventricle is noticeably enlarged, have the greatest risk of sudden heart failure. How is it diagnosed? Children with aortic valve stenosis usually have a heart murmur that can be heard with a stethoscope. A doctor can also detect pulse abnormalities and will see irregularities on an electrocardiogram (ECG). A chest x-ray may also show an enlarged left ventricle. An echocardiogram may also be ordered. This technique uses ultrasound waves to create an image of the heart and its function. In cases where severe blockage is suspected and surgery most likely, doctors may do a left heart cardiac catheritization. In this procedure, a thin tube (catheter) is inserted through an artery and moved into the heart chambers. This procedure will measure the pressure difference between the left ventricle and the aorta.
What is the treatment?
Children with aortic valve stenosis require close medical supervision and should undergo yearly exercise testing after the age of six. Most children with mild to moderate aortic valve stenosis can engage in normal physical activities. Surgery is only performed in cases of severe obstruction. The most common treatments are surgical valve repair and balloon valvuloplasty. A balloon-tipped catheter is inserted into the aortic valve. The balloon is then inflated to expand the valve opening. Some forms of aortic valve stenosis require medical management with beta-blockers or calcium channel blockers.
Self-care tips
If your child has been diagnosed with aortic valve stenosis, follow your doctor’s recommendations for treatment and physical activity.