How is Supravalvular aortic stenosis treated?

Surgery is the primary treatment for supravalvar aortic stenosis (SVAS). Children and adolescents with catheter peak-to-peak (or Doppler mean) gradient of 50 mm Hg or more should have surgical intervention. The choice of procedures in these patients is similar to that indicated for valvar aortic stenosis.

How serious is narrowing of the aorta?

Aortic stenosis is one of the most common and serious valve disease problems. Aortic stenosis is a narrowing of the aortic valve opening. Aortic stenosis restricts the blood flow from the left ventricle to the aorta and may also affect the pressure in the left atrium.

Is Aortic Stenosis a genetic disease?

A single, specific genetic cause of aortic valve stenosis (AVS) has not been identified. Some fetuses diagnosed with AVS will eventually progress to having hypoplastic left heart syndrome (HLHS). Those with severe AVS can also develop endocardial fibroelastosis, depending on the time of onset during fetal development.

What kind of murmur is aortic stenosis?

Stenosis of the aortic or pulmonic valves will result in a systolic murmur, as blood is ejected through the narrowed orifice.

Which syndrome is associated with Supravalvular aortic stenosis?

Supravalvular aortic stenosis (SVAS) can either be isolated or can occur as part of Williams syndrome.

How common is subaortic stenosis?

Subvalvular aortic stenosis is a rare disorder seen in infants and newborns but is the second most common type of aortic stenosis. It is responsible for approximately 1% of all congenital heart defects (8 in 10,000 births) and 15% to 20% of all fixed left ventricular outflow tract obstructive lesions.

Does aortic stenosis run in families?

Although epidemiologic studies cannot directly infer causality, our results suggest the existence of a familial component to the disease. Our results suggest that patients with ischaemic heart disease and a family history of aortic stenosis have a high relative risk of aortic stenosis.”

What is Williams-Beuren syndrome?

Williams syndrome, also known as Williams-Beuren syndrome, is a rare genetic disorder characterized by growth delays before and after birth (prenatal and postnatal growth retardation), short stature, a varying degree of mental deficiency, and distinctive facial features that typically become more pronounced with age.

¿Cuáles son los síntomas de la estenosis de la válvula aórtica?

En general, los signos y síntomas de la estenosis de la válvula aórtica se manifiestan cuando el estrechamiento de la válvula es grave. Es posible que algunas personas que padecen estenosis de la válvula aórtica no tengan síntomas durante muchos años. Los signos y síntomas de la estenosis de la válvula aórtica pueden ser:

¿Qué es la estenosis aórtica?

I. ESTENOSIS VALVULAR AORTICA La estenosis aórtica (EA) es en una mal-formación de la válvula aórtica, de carác-ter progresivo, que produce una obstruc-ción al flujo de salida del ventrículo iz-quierdo. La válvula puede ser unicúspide (típica de la estenosis critica del recién nacido, en la que la válvula se parece más a una masa

¿Por qué la válvula aórtica se obstruye?

Este estrechamiento impide que la válvula se abra por completo, lo que reduce u obstruye el flujo sanguíneo del corazón a la arteria principal del cuerpo (aorta) y hacia el resto del organismo. Cuando el flujo de sangre que pasa por la válvula aórtica se reduce o se obstruye, el corazón debe trabajar más para bombear sangre al cuerpo.

¿Cómo nacen los niños con una válvula aórtica?

Algunos niños nacen con una válvula aórtica que tiene solo dos válvulas (bicúspide) en lugar de tres. Algunas personas también pueden nacer con una valva (unicúspide) o con cuatro valvas (cuadricúspide), pero no es frecuente.