Subvalvular aortic stenosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editors-In-Chief: Claudia P. Hochberg, M.D. [2]; Abdul-Rahman Arabi, M.D. [3]; Keri Shafer, M.D. [4]
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Epidemiology and Demographics
Aortic subvalvular stenosis is the second most common level of congenital obtruction of LV outflow, located just beneath the aortic valve and occurs in 8-30% of all forms of left ventricular outflow tract obstruction. IHSS is not present at birth and is not considered a congenital lesion.
The lesion is caused by accumulation of fibrous elastic tissue which most often
Anatomy
There are several varieties of Congenital Aortic Subvalvular Stenosis (or subaortic stenosis):
- Membranous: A fixed localized membrane 0.5 to 2 cm below the level of the aortic valve and attached to the septum and the base of the anterior mitral leaflet.
- Fibromuscular:
- More commonly there is a fibromuscular membrane or tunnel with a significant muscular component which can sometimes be hard to distinguish from IHSS. This is a more severe form and is often associated with a small aortic root.
- Associated aortic insufficiency (AI) is often present due to the high speed jet of blood through the aortic cusps resulting in fibrosis and retraction.
- Congenital anomalies of the mitral valve:
- Attachment to ventricular septum of accessory chordae from anterior mitral leaflet
- Redundant AV valve tissue causing subaortic obstruction.
- Aneurysm of the membranous ventricular septum
Clinical Features
- Similar to that of valvular aortic stenosis.
- AI more common in this form (50 to 75% of patients).
- Symptoms begin in infancy or early adulthood.