Last Updated Dec 2008

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Aortic Stenosis

Etiology
Caused by congenital malformation of the aortic valve, resulting in thickened, fused
valve leaflets with restricted systolic movement and variable degrees of left ventricular
outflow tract obstruction
As is most commonly due to thickening of a congenitally bicuspid valve
Congenital left ventricular outflow tract obstruction may also be secondary to isolated or
associated subvalvular or supravalvular aortic stenosis
Acquired AS secondary to rheumatic heart disease is rare before adulthood
Left ventricular hypertrophy and its complications ensue if untreated.
Epidemiology
Congenital AS represents 5% of all congenital heart disease
3:1 male to female predominance
Bicuspid aortic value is common, occurring in 2% of population.
Signs/Symptoms
Harsh systolic murmur
Located at right upper sternal border
Radiates to the neck
+ decreased first heart sound
The louder, harsher, and longer the murmur, the more severe the stenosis
Symptoms vary with degree of stenosis
Mild and moderate cases are generally asymptomatic, even during activity
Sever cases: Chest pain, fatigue, dizziness, or syncope during activity
Critical AS presents in early infancy with evidence of CHF / cardiogenic shock, including
shock, including decreased cardiac output and pulmonary edema.
Treatment
Balloon valvotomy
Has emerged as the initial treatment of choice in aortic valve stenosis
Not useful in subvalvular or supravalvular AS
Prosthetic valve replacement
Attempt to avoid this procedure in children, to avoid need for anticoagulation
Neonatal patients with critical AS require medical intervention prior to attempted surgical
intervention or balloon valvotomy
Prostaglandin E1
Inotropic agents
Ventilator support
Correction of acidosis
Differential Dx
Aortic valve stenosis
Subvalvular aortic stenosis
Supravalvular aortic stenosis
Hypoplastic left ventricle
Cardiomyopathy (hypertrophic or dilated).
Diagnosis
Clinical examination
EKG: left ventricular hypertrophy
CXR: Normal heart size, prominent ascending aorta
Echocardiography (with Doppler) is diagnostic; also defines severity
Cardiac catheterization can further define anatomy and confirm severity of aortic
gradient; may also allow for intervention with balloon aortic valvotomy in cases of severe
AS.
Prognosis/Clinical Course
Prognosis varies with severity of lesion
Some severe cases die in the neonatal period; those who respond well to surgery may
require reoperation later in life
Mild to moderate cases should be followed for progression of stenosis; patients who
require valve replacement in later decades generally have a very good prognosis
Limitation of physical activity recommended in some cases.