Aortic Stenosis

Aortic stenosis (AS), or the narrowing of the aortic valve aperture, is the most common valvular
heart disease. While rheumatic heart disease remains the most frequent etiology worldwide,
degenerative AS and congenital bicuspid valve defect are the 2 usual causes in developed
countries. Aortic stenosis gradually progresses to heart failure, producing exertional dyspnea,
angina, and/or syncope. A crescendo-decrescendo systolic murmur is audible in the right upper
sternal border. Doppler echocardiography is the imaging of choice, showing structural and flow
changes in the valvular area. Valve replacement is the only effective treatment for symptomatic
severe AS. Indications for the procedure depend on the patient's symptoms, degree of AS
severity, exercise tolerance, concurrent cardiac abnormalities, surgical risk, and life expectancy.

Last updated: July 6, 2023

CONTENTS

Overview
Pathophysiology
Clinical Presentation
Diagnosis
Management
Differential Diagnosis
References

Overview
Definition
Aortic stenosis (AS) is the narrowing of the aortic valve aperture.
 Causes varying degrees of blood flow obstruction between the left ventricle (LV) and the
aorta
Consequently, produces left ventricular pressure overload
If left untreated, results in left ventricular dysfunction and heart failure

Epidemiology
Most common valvular heart disease
Rheumatic heart disease: most common cause of AS worldwide
In developed countries, most common causes are:
Calcific aortic valve disease
Congenital bicuspid aortic valve (affects 1%–2% of the population)
Prevalence increases with age.
Men > women

Etiology
Senile calcific AS:
Also known as degenerative AS
Dystrophic calcification of the aortic valve
Most common cause in adults > 70 years in developed countries
Risk factors:
Hypertension
Diabetes
Smoking
End-stage kidney disease
Disturbances in mineral metabolism
Rheumatic heart disease:
Most common cause in the developing world
Often associated with mitral stenosis
Presents in the 3rd to 5th decade of life
Congenital bicuspid aortic valve:
Most common congenital heart valve defect
More predominant in men
Most common cause in adults < 70 years in developed countries
Predisposes to early calcification and subsequent stenosis
Can present with other cardiac abnormalities (e.g., coarctation of the aorta)
Radiation
Unicuspid valve
Pathophysiology
Mechanisms of valvular damage
Senile calcific AS:
Hemodynamic stress results in progressive valve thickening and damage, which
predispose to calcification.
Risk factors and calcification of the leaflet bodies → aortic valve stenosis and
obstruction
Gross morphologic hallmark: calcified masses on the outflow cusp surfaces (which limit
full opening)
Rheumatic heart disease:
Inflammation of the valve causes necrosis and injury.
Postinflammatory fibrosis of the valve leaflets leads to commissural fusion →
calcification and stenosis
Bicuspid valve:
2 cusps, which are often of unequal size
Malformed valves contribute to turbulence of blood flow, which eventually leads to
calcific degeneration.

Mechanism of heart failure

Normal aortic valve opening: cross-sectional area approximately 3–4 cm²
Change in gradient noted when the orifice area is < ½ of normal
Gradual progression of stenosis allows adaptive changes.
Obstruction of transvalvular blood flow → increased left ventricular pressure to maintain
cardiac output (CO) → Left ventricular hypertrophy (LVH) occurs, in order to overcome the
increased afterload.
Hypertrophic left ventricle leads to decreased compliance → ↑ left
ventricular end diastolic pressure (LVEDP)
LVH, ↑ LVEDP, and continuous valvular obstruction increase LV oxygen demand → ↑
ischemia and myocardial fibrosis
Changes result in decreased contractility and CO → heart failure.
Other complications:
Arrhythmias (e.g., atrial fibrillation)
Pulmonary hypertension
Image demonstrates the relationship between left ventricular pressure (green line) and aortic pressure (red
line) throughout the cardiac cycle. The circle corresponds with the point at which the aortic valve would
normally open, beginning the ventricular ejection phase. In aortic stenosis, left ventricular pressure exceeds
the aortic pressure to overcome the stenotic valve. This leads to a systolic ejection murmur.
Image by Lecturio.

Clinical Presentation
Symptoms
 Asymptomatic for years
Exertional dyspnea and fatigue (initial symptoms)
Classic triad (late in the course of the disease):
1. Exertional angina or chest pain (from ischemia)
2. Syncope:
On exertion (exercise-induced vasodilation, under the setting of obstruction with
fixed CO, leads to hypotension)
At rest (from associated arrhythmias)
3. Left heart failure:
Orthopnea
Paroxysmal nocturnal dyspnea
Pulmonary edema

Signs
Unremarkable early in the course
Pulse and blood pressure:
Narrow pulse pressure with severe AS
Pulsus parvus et tardus (low-amplitude (parvus), delayed (tardus) peak in the carotid
arterial pulse)
Cardiac inspection and auscultation:
Hyperdynamic left ventricle leading to heave with a double or laterally displaced apical
impulse
Heart sounds may have:
Single soft S2 (aortic valve closure diminished or absent; synchronous A2P2)
Paradoxical splitting of S2 (pulmonic valve closure, P2, is followed by aortic valve
closure, A2)
S4: reflects a strong atrial contraction against LVH
Ejection click in children and young adults (congenital AS)
Murmur:
Systolic low-pitched crescendo-decrescendo murmur heard best in the right
upper sternal border and radiating to the carotids
Gallavardin effect: occasional downward radiation of AS murmur to the cardiac
apex (may be confused with mitral regurgitation murmur)
Provocative maneuvers:
Decreased murmur: Valsalva maneuver, standing (decreased intracardiac volume)
or sustained handgrip (increased afterload)
Increased murmur: leg raising, squatting, and after a premature ventricular beat
(increased preload)
 Phonocardiograms of abnormal heart sounds caused by the following cardiac defects:
aortic regurgitation, mitral valve prolapse, mitral stenosis (MS), aortic stenosis (AS), tricuspid regurgitation,
hypertrophic obstructive cardiomyopathy (HOCM), atrial septal defect (ASD), ventricular septal defect (VSD),
and patent ductus arteriosus (PDA)
Image by Lecturio.

Audio:

This audio clip is an example of severe aortic stenosis. This is a harsh, crescendo-
decrescendo murmur occurring between S1 and S2. Due to the severity of the aortic stenosis
, the S2 heart sound is inaudible.

0:00 / 1:01

Heart sound
(https://www.med.umich.edu/lrc/psb_open/html/repo/primer_heartsound/primer_heartsound.html) by The
Regents of the University of Michigan. License: CC BY-SA 3.0 (https://creativecommons.org/licenses/by-
sa/3.0/)
Diagnosis
Transthoracic and Doppler echocardiography
Diagnostic imaging of choice
Details provided:
Structure and function of the valve
Valvular findings: decreased aortic valve opening, valvular calcification, bicuspid valve
Other findings: LV hypertrophy, aortic wall abnormalities, systolic and diastolic function
Systolic dysfunction or LV ejection fraction (EF) < 50%: important in determining AS
management
Parameters of stenosis (from Doppler echocardiography):
Transvalvular gradient (in mm Hg)
Flow velocity with decreased valvular area
Aortic valve area (in cm²):
Severe AS: valve area < 1 cm2
Moderate AS: valve area 1–1.5 cm2
Mild AS: valve area 1.5–2 cm2
Additional information provided:
Other coexisting valvular disease
Hypertrophic cardiomyopathy
Ascending aortic aneurysm associated with bicuspid valves
Coarctation of aorta

Other tests
ECG:
Results vary and depend on associated structural abnormalities and arrhythmias.
May demonstrate left ventricular hypertrophy, left atrial enlargement, and left axis
deviation
Chest X-ray:
On lateral views, aortic valve calcification can be seen.
Pulmonary congestion from LV failure can be visualized.
Exercise stress testing or exercise tolerance test (ETT):
Contraindicated in symptomatic severe AS
Performed in select cases (e.g., unclear level of physical activity, low-gradient AS)
Cardiac catheterization and coronary arteriography:
Provides information on the pressure gradient between the left ventricle and aorta, and
overall hemodynamic assessment when other tests are inconclusive
Evaluation of coronary artery disease: an important consideration prior to valvular
surgery
Multidetector computed tomography (MDCT):
Helps in evaluation of low-gradient AS
Quantification of aortic valve calcification correlates with severity of stenosis.
Management
General principles
 Aortic valve replacement (AVR): mainstay of treatment of symptomatic AS
To determine candidates for AVR, the following details are obtained:
Symptom status
Severity of AS:
Severe AS: aortic jet velocity ≥ 4 m/sec, mean transvalvular gradient of ≥ 40 mm
Hg
Severe AS: typically with < 1 cm² area (but is not required)
LV systolic function: LVEF < 50% = systolic dysfunction
Indications for AVR:
Asymptomatic severe AS:
Abnormal ETT (decreased exercise tolerance)
With LVEF < 50%
Undergoing other cardiac surgery
Rapid disease progression and low surgical risk
Symptomatic severe AS
For less severe or moderate AS, performing AVR depends on multiple factors:
Symptoms
Aortic valve area < 1 cm²
LVEF
ETT, dobutamine stress echocardiography
If undergoing other cardiac surgery

Surgical management
For patients with life expectancy > 1 year and if surgery is likely to improve quality of life
Transcatheter aortic valve implantation (TAVI):
In patients with intermediate-high surgical risk
In patients with low surgical risk meeting feasibility for TAVI
Surgical aortic valve replacement (SAVR):
In patients with low or intermediate risk and TAVI is not feasible
Presence of another indication for cardiac surgery
Percutaneous aortic balloon valvuloplasty (PABV):
Not a substitute for valve replacement
Has high rate of re-stenosis
Consider as a bridge to TAVI or SAVR in severe symptomatic AS
Palliation for patients who are not good candidates for AVR
Has a role for young and adolescent patients

General recommendations for non-surgical management

 Periodic monitoring:
TTE every 6–12 months in asymptomatic severe AS
TTE every 1–2 years for moderate AS and every 3–5 years for mild AS
Activity:
Avoid strenuous physical activity and competitive sports in severe AS.
Avoid dehydration (to protect against CO reduction).
Medications:
Endocarditis prophylaxis: with history of infective endocarditis
General guidelines: Start with low dose and slowly titrate.
Angiotensin-converting enzyme (ACE) inhibitors: may improve LV fibrosis in addition to
blood pressure control
Beta blockers:
Reduce contractility
Continue with coexisting coronary artery disease or atrial fibrillation
For hypertension, consider a low dose but generally avoid with symptomatic AS
and heart failure.
Diuretics:
Excessive decrease in preload should be avoided.
Use with caution if the patient has symptomatic AS (because it reduces
ventricular volume).

Prognosis
If untreated with surgery, 50% of patients die within 3 years of symptom onset (angina,
syncope, or heart failure).
Surgical mortality rate for valve replacement including the elderly is 2%–5%.

Differential Diagnosis
 Hypertrophic cardiomyopathy: a cardiac condition characterized by a thickened left
ventricular wall leading to left ventricular outflow obstruction and diastolic dysfunction.
Patients present with exertional dyspnea, syncope, and, in extreme cases,
sudden cardiac death. The systolic murmur increases with the Valsalva maneuver and
standing. Diagnosis is by echocardiography. Management includes negative inotropic agents
with surgical options to reduce the LV outflow obstruction. An implantable defibrillator is
placed for those at risk for sudden death.
Myocardial infarction: an acute blockage of the coronary arteries supplying the heart, which
can predispose to structural valvular incompetence. This condition commonly presents with
chest pain and is diagnosed with ECG and cardiac enzyme tests.
Aortic regurgitation: characterized by the backflow of blood from the aorta to the left
ventricle, commonly caused by rheumatic heart disease, and congenital and degenerative
valvular disorders. Examination shows an early diastolic high-pitched decrescendo murmur,
heard best in the left sternal border. Diagnosis is by echocardiography. The acute form is a
medical emergency requiring immediate surgery.
Heart failure (HF) with reduced ejection fraction: a chronic, progressive condition
characterized by left ventricular dysfunction from impaired myocyte contractility. This leads to
subsequent volume overload. Risk factors include hypertension, coronary artery disease, and
diabetes mellitus. Heart failure can develop with or without valvular abnormalities.

References
1. Ahmed, S., & Bernath, G.A. (2017). Valvular heart diseases. Elmoselhi, A. (Ed.), Cardiology: An Integrated
Approach. McGraw-Hill.
2. Bashore, T.M., & Granger, C.B., & Jackson K.P., & Patel M.R. (2021). Aortic stenosis. Papadakis M.A., & McPhee
S.J., & Rabow M.W.(Eds.), Current Medical Diagnosis & Treatment 2021. McGraw-Hill.
3. Gaasch, W., Otto, C., & Yeon, S. (2020) Natural history, epidemiology, and prognosis of aortic stenosis.
UptoDate. Retrieved 31 Oct 2020, from https://www.uptodate.com/contents/natural-history-epidemiology-and-
prognosis-of-aortic-stenosis (https://www.uptodate.com/contents/natural-history-epidemiology-and-prognosis-
of-aortic-stenosis)
4. Grimard, B., Safford, R., & Burns, E. (2016) Aortic stenosis: Diagnosis and Treatment. Am Fam Physician. 2016
Mar 1;93(5):371-378.
5. Mitchell, R., & Halushka, M. (2020) Blood vessels. In Kumar, V., Abbas, A., Aster, J. & Robbins, S. Robbins and
Cotran Pathologic Basis of Disease (10th Ed., pp. 557-567). Elsevier, Inc.
6. O’Gara, P.T., & Loscalzo, J. (2018). Aortic valve disease. Jameson J, & Fauci A.S., & Kasper D.L., & Hauser S.L., &
Longo D.L., & Loscalzo J(Eds.), Harrison's Principles of Internal Medicine, 20e. McGraw-Hill.
7. Otto, C., Cooper, S., Gaasch, W., & Yeon, S. (2019) Medical management of symptomatic aortic stenosis.
UpToDate. Retrieved 1 Nov 2020, from https://www.uptodate.com/contents/medical-management-of-
symptomatic-aortic-stenosis (https://www.uptodate.com/contents/medical-management-of-symptomatic-aortic-
stenosis)
8. Otto, C., Gaasch, W., & Yeon, S. (2019) Medical management of symptomatic aortic stenosis. UpToDate.
Retrieved 1 Nov 2020, from https://www.uptodate.com/contents/medical-management-of-asymptomatic-aortic-
stenosis-in-adults (https://www.uptodate.com/contents/medical-management-of-asymptomatic-aortic-stenosis-
in-adults)
9. Ren, X., & O-Brien, T. (2019) Aortic stenosis, Medscape. Retrieved 31 Oct 2020, from
https://emedicine.medscape.com/article/150638-overview#a5 (https://emedicine.medscape.com/article/150638-
overview#a5)