Subvalvular aortic stenosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Epidemiology and Demographics

Aortic subvalvular stenosis is the second most common form of congenital left ventricular outflow tract obtruction and occurs in 8-30% of all forms of left ventricular outflow tract obstruction. The level of obstruction is located just beneath the aortic valve. HOCM (a.k.a. idiopathic hypertrophic subaortic stenosis or IHSS) is not present at birth and is not considered a congenital lesion.

Pathophysiology

There are several varieties of Congenital Aortic Subvalvular Stenosis (or subaortic stenosis):

1. 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.
2. 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.
3. Congenital anomalies of the mitral valve:
   • Attachment to ventricular septum of accessory chordae from anterior mitral leaflet
   • Redundant AV valve tissue causing subaortic obstruction.
4. Aneurysm of the membranous ventricular septum

Epidemiology and Demographics

Aortic insufficiency is more common in this form of aortic stenosis and occurs in 50 to 75% of patients.

Natural History

The symptoms are similar to that of valvular aortic stenosis and but begin earlier in infancy or early adulthood.

Diagnosis

Symptoms

The symptoms are similar to that of valvular aortic stenosis and but begin earlier in infancy or early adulthood.

Treatment

The membrane can be resected as shown here: