Supravalvular aortic stenosis (SVAS) is a medical condition characterized by a narrowing or constriction of the aorta, specifically the area above the aortic valve. The aorta is the main artery that carries oxygen-rich blood from the heart to the rest of the body. In individuals with SVAS, the narrowing occurs in the region just above the aortic valve, where the aorta originates from the left ventricle of the heart.
This narrowing of the aorta can obstruct the blood flow, causing the heart to work harder in order to pump blood through the constricted area. The severity of the condition can vary, ranging from mild to severe, and may progress over time. In some cases, SVAS can be present at birth (congenital), while in others, it may develop later in life (acquired).
Symptoms of supravalvular aortic stenosis can include chest pain, shortness of breath, fatigue, dizziness, and fainting. However, some individuals may remain asymptomatic.
The causes of SVAS can be genetic in nature, linked to a condition known as Williams syndrome. In this syndrome, various developmental abnormalities occur due to a specific genetic mutation. However, SVAS can also occur in individuals without this syndrome, and the exact cause may be unknown.
Treatment options for SVAS depend on the severity of the stenosis and the presence of symptoms. Mild cases may not require any specific intervention, while more severe cases may necessitate surgical intervention, such as balloon angioplasty, stent placement, or even aortic valve replacement. Regular monitoring and follow-up with a cardiologist are typically recommended for individuals with SVAS.
