Supravalvular Aortic Stenosis
What is Supravalvular Aortic Stenosis?
Supravalvular aortic stenosis (SVAS) is a rare congenital heart condition that is caused by the constriction of the aortic valve and the vessels above it. It affects the aortic valve most specifically, the part of the aorta that branches off from the aortic valve. When SVAS is present, the diameter of the affected portion of the aorta narrows so that not enough blood can flow through it.
The Symptoms and Diagnosis of Supravalvular Aortic Stenosis
The symptoms of SVAS depend on the severity of the stenosis and the age of the patient. Common symptoms include fatigue and tiredness, chest pain, shortness of breath, and paleness. In some cases, a heart murmur may be heard when listening to the heartbeat. If left untreated, SVAS can lead to complications such as congestive heart failure, valve leakage, or aortic regurgitation.
SVAS is usually diagnosed using echocardiography, CT angiography, or cardiac MRI. An electrocardiogram (ECG) and chest X-ray may also be taken to look for abnormal electrical patterns in the heart. Furthermore, some genetic tests may be done to aid in the diagnosis of the condition. After diagnosis, treatment options should be discussed with the doctor.
Treatment for Supravalvular Aortic Stenosis
The primary goal of treatment is to improve the blood flow through the narrowed aorta. Treatment options for SVAS include:
- Surgery – Aortic balloon valvuloplasty is a procedure specifically used to open up the narrowed valve by inserting a balloon-shaped device into the aortic valve and inflating it. This procedure is often used to treat children who are too young for open-heart surgery.
- Medication – Some medications, such as beta-blockers and ACE inhibitors can help reduce the symptoms and improve the blood flow through the narrowed aorta.
- Open-heart surgery – In severe cases, open-heart surgery may be necessary to remove the narrowed section of the aorta.
SVAS can be a serious condition, but with the right treatment plan, it can be managed. It is important to work closely with your doctor to ensure that the treatment plan is tailored to your individual needs.