II. Epidemiology
- Overall Prevalence in U.S. 3.7%
- Aortic Stenosis is the most significant cardiac valve disorder in the developed world
-
Prevalence increases with age (U.S.)
- Age 50-60 years: 0.2%
- Age 60-70 years: 1.3%
- Age 70-80 years: 3.9%
- Age 80-90 years: 9.8%
III. Causes: Valvular
- Congenital Bicuspid Valve (Most common)
- Affects 1-2% of the U.S. population
- Twice as common in men
- Also associated with aortic root dilation
- Slow increase in stenosis (progressive sclerosis)
- Childhood: mild stenosis and asymptomatic
- Ages 20-40: moderate stenosis develops
- Over age 40: severe stenosis develops (occurs in >50%, requiring aortic Valve Replacement)
- Atherosclerosis (Calcific Aortic Valve Stenosis)
- Gradually develops, typically presenting over age 70 years
- Rarely severe (contrast with congenital )
- Similar pathway to atherosclerosis with valvular Plaque accumulation and inflammation
- Calcification occurs via Inflammatory response to LDL Cholesterol and Lipoprotein-A deposition
- Calcification affects normal aortic valves despite having 3 leaflets (tricuspid, in contrast to congenital bicuspid valve)
- Calcification of the aortic valve leads to aortic sclerosis with tissue rigidity
- Aortic sclerosis is a calcification of the aortic valve WITHOUT restriction of valve leaflet mobility
- Aortic sclerosis progresses to Aortic Stenosis in 2% of patients per year
- Aortic Stenosis gradually progresses to restrict the aortic valve opening
- Gradually develops, typically presenting over age 70 years
-
Rheumatic Fever
- Slowly progressive stenosis
- Subacute Bacterial Endocarditis
- Other acquired aortic valve stenosis causes
- Other congenital aortic valve stenosis causes
- Aortic Coarctation
- Unicuspid aortic valve
- Very rare congenital defect presenting with Aortic Stenosis at a young age
- May be initially misdiagnosed as a bicuspid valve
- Williams Syndrome
- Shanes Complex
- Supravalvular Aortic Stenosis
- May be associated with Williams Syndrome or other congenital supravalvular Aortic Stenosis
- Subvalvular Aortic Stenosis
- May be associated with Hypertrophic Cardiomyopathy or other congenital subvalvular Aortic Stenosis
- References
- Baloor and Nayak (2018) Exam Preparatory Manual for Undergraduate Medicine, Jaypee Brothers Medical Publication
IV. Pathophysiology: Course
- Initial: Long asymptomatic latent period
- Course
- Aortic Stenosis gradually progresses to restrict the aortic valve opening
- Increased Left Ventricular Outflow Obstruction and flow restriction with increased left ventricular pressures
- Next: Concentric Left Ventricular Hypertrophy (left ventricular wall thickening with preserved LV volume)
- Next: Diastolic Dysfunction (resistance to LV filling with preserved systolic function)
- Next: Systolic Dysfunction and Congestive Heart Failure
- Next: Increased myocardial oxygen demand, Coronary Artery compression and secondary Angina
- Next: Hypotension and Syncope in response to Exercise
V. Risk Factors
- Age >65 years old
- Male gender
- Bicuspid aortic valve (affects 1-2% of U.S. population)
- Other specific valvular conditions (see causes above)
- Cardiovascular disease and associated risk factors
- Hypertension (Hazard Ratio 1.7 for severe Aortic Stenosis)
- Diabetes Mellitus (Hazard Ratio 1.5 for severe Aortic Stenosis)
- Tobacco Abuse
- Hyperlipidemia (Hazard Ratio 1.2 for severe Aortic Stenosis)
- Lipoprotein-A or Lp(a) level elevated (genetic factor; Odds Ratio 1.2 for Aortic Stenosis)
- Miscellaneous
- Thoracic radiation history
- Rheumatologic Conditions (e.g. SLE, RA)
- References
VI. Symptoms
- Mild to Moderate stenosis
- Asymptomatic for a long latent period while stenosis progresses
- More subtle symptoms may go unrecognized
- Fatigue
- Exercise intolerance
- Light Headedness
- May present when unmasked by acute comorbidity that exacerbates occult Aortic Stenosis
- Severe Obstruction
- Dyspnea (most common presenting symptom)
- Other presenting symptoms
VII. Signs
- Precautions
- Lack of cardiac murmur has an 88% Negative Predictive Value for significant valvular disease
- Exam is otherwise unreliable in detecting valvular heart disease (compared with Echocardiography)
- Test Sensitivity: 44%
- Test Specificity: 69%
- References
- Classic Murmur
- Harsh, late-peaking, crescendo-decrescendo Systolic Murmur
- Medium pitch
- Heard best at right upper Sternum (second intercostal space)
- May also be heard at apex (esp. elderly)
- May radiate into bilateral Carotid Artery region (LR+ 12)
- Mild Aortic Stenosis
- Loud ejection click (best heard at apex)
- Short, early Systolic Murmur (at right second intercostal space)
- Loud A2 heart sound (best heard at aortic area)
- Moderate Aortic Stenosis
- Ejection click (best heard at apex)
- Early Systolic Murmur (loudest at right second intercostal space)
- Transmitted to Supraclavicular, Carotids, Apex
- Harsh
- Systolic ejection murmur that peaks later in systole
- Ends well before A2 heart sound
- Arterial Pulse altered
- Upstroke of the pulse has shudder
- Delayed, prolonged, low-volume carotid pulsation (Pulsus parvus et tardus)
- Test Sensitivity 70% and Test Specificity 98% in Aortic Stenosis
- Roldan (1996) Am J Cardiol 77(15): 1327-31 [PubMed]
- Apex impulse may be abnormal, accentuated
- Slightly sustained
- Presystolic Shoulder ("a wave") precedes major systolic impulse
- Systolic thrill may be palpated at base
- S4 Gallup Rhythm
- Severe Aortic Stenosis
- Ejection click NO longer present
- A2 heart sound is markedly diminished or inaudible (LR+ 15.7)
- Systolic Murmur
- Variable loudness (may be quiet despite severity)
- Long, nearly holosystolic
- Harsh (especially at aortic area)
- Carotid Pulse very abnormal, with a delayed upstroke (LR+ 6.3)
- Very slow and long upstroke (Pulsus parvus et tardus)
- Overall very weak pulse
- Brachioradial delay
- Right Brachial Pulse and right Radial Pulse are simultaneously palpated
- Radial Pulse is felt after the Brachial Pulse in severe Aortic Stenosis (also in MR with severe CHF)
- Apical impulse abnormal
- Strong and sustained for all of systole
VIII. Signs: Most significant findings
- Precautions
- Pulse changes may be masked by atherosclerosis or Hypertension
- Murmur may be less prominent with reduced LV function
- Murmur may radiate to apex but not carotids in elderly
- High Positive Likelihood Ratio (rule-in diagnosis)
- Pulsus parvus et tardus (low pulse volume and slow rate of rise of carotid or Brachial Pulse)
- Low Negative Likelihood Ratio (most likely to rule-out diagnosis)
- Absence of late peaking murmur (early peaking murmur is typically benign)
- Lack of radiation to right carotid or clavicle
- Normally split Second Heart Sound (S2)
IX. Differential Diagnosis
- See Heart Murmur
- See Dyspnea Causes
- See Syncope
- Supravalvular Aortic Stenosis
- Membranous supravalvular Aortic Stenosis
- Hypertrophic Cardiomyopathy (IHSS)
- Mitral Regurgitation
X. Associated Conditions
- Atrial Fibrillation (17% of Aortic Stenosis patients)
- Coronary Artery Disease (24 to 68% of Aortic Stenosis)
- Heyde Syndrome
- Rare Coagulopathy resulting from acquired Von Willebrand Disease
- Von Willebrand Factor (VVWF) is fragmented by Aortic Stenosis, resulting in VWF Deficiency
- Presents with Gastrointestinal Bleeding and an Aortic Stenosis murmur
- Coagulopathy improves with aortic Valve Replacement
- Bicuspid aortic valve associated conditions
- Aortic Root Dilation
- Aortic Dissection
- AV Node Block
XI. Diagnotics: Electrocardiogram
- Precautions
- Electrocardiogram may only be abnormal in moderate to severe Aortic Stenosis
- Common findings
- Left atrial enlargement
- Left Ventricular Hypertrophy
- Peak systolic gradient (PSG) has been correlated to the QRS amplitude (LVH criteria)
- Kishore (1990) Indian Heart J 42(1): 62-5 [PubMed]
- Other findings
- T Wave reduction in leads I, avL, V5, V6
- Left Anterior Hemiblock or Left Bundle Branch Block
- Complete AV Block
XII. Imaging
-
Chest XRay
- Precautions
- Chest XRay is a low yield test in Aortic Stenosis diagnosis
- Chest XRay is primarily used to evaluate presenting symptoms (Dyspnea, Syncope, Chest Pain)
- Findings (unreliable)
- Apical Contour abnormal suggests large left ventricle
- Prominent ascending aorta
- Aortic valve calcification
- Left Ventricular Hypertrophy may appear as a boot-shaped heart
- Precautions
-
Echocardiogram
- Most important study in the evaluation of suspected Aortic Stenosis
- Frequency
- Initial presentation
- Once Aortic Stenosis is diagnosed, repeat echo per monitoring schedule based on severity (see below)
- Indications
- Loud (grade 3), unexplained Systolic Murmur (esp. holosystolic, late systolic)
- Single Second Heart Sound
- History of bicuspid aortic valve
- Symptoms suggestive of Aortic Stenosis
- New murmur associated with new symptom presentation (Dyspnea, Syncope, Angina)
- Findings
- Aortic Stenosis diagnosis
- Aortic Stenosis grading with aortic valve gradient, orifice size, jet velocity (see classification above)
- Aortic valve thick and sclerotic (bright white) with poor excursion on PLAX View
- Continuous Wave Doppler (CWD) at aortic valve with jet velocity measured in A5C View
- Aortic Stenosis complications (LV hypertrophy, Diastolic Dysfunction, Systolic Dysfunction)
- Aortic Stenosis grading with aortic valve gradient, orifice size, jet velocity (see classification above)
- Other aortic valve disorders
- Bicuspid aortic valve
- Mildly obstructed valve
- Thickened, sclerotic valve (aortic sclerosis)
- Other valve disorders presenting similarly to Aortic Stenosis (Dyspnea, Syncope, Angina)
- Acute Mitral Regurgitation
- May be seen with Myocardial Infarction with papillary Muscle rupture
- Chronic Mitral Regurgitation may be associated with Aortic Stenosis (worse prognosis)
- Prosthetic valve disorders (e.g. valvular regurgitation or obstruction)
- Acute Mitral Regurgitation
- Aortic Stenosis diagnosis
- Cardiac Catheterization (Angiogram)
- Can directly measure left ventricular pressure gradient
XIII. Classification: Aortic Stenosis Severity (by Echocardiogram)
- Aortic valve area (AVA in cm2)
- Normal: 3 to 4 cm2
- Mild: 1.5 to 2 cm2
- Moderate: 1 to 1.5 cm2
- Severe: <1 cm2
- Critical: <0.8 cm2
- Low Flare, Low Gradient Severe Aortic Stenosis <1 cm2
- But Vmax <4 m/sec and mAVPG <40 mmHg due to HFrEF with decreased Stroke Volume
- Maximum Aortic jet velocity (Vmax in m/sec, transaortic valve velocity)
- Normal: <2.0 m/sec
- Mild: 2.0 to 2.9 m/sec
- Moderate: 3.0 to 3.9 m/sec
- Severe: >4.0 m/sec
- Mean Aortic Valve Pressure Gradient (mAVPG in mmHg)
- Normal: <10 mmHg
- Mild: 10-20 mmHg
- Moderate: 20-40 mmHg
- Severe: >40 mmHg
- Critical: >50 mmHg
XIV. Management: General Measures
- Asymptomatic Aortic Stenosis progression is not prevented by any specific measures
- SBE Prophylaxis is no longer recommended (until aortic Valve Replacement, or history of prior endocarditis)
- Manage comorbid conditions
- Maintain adequate hydration (Preload dependent)
- Avoid strenuous Exercise or activity in moderate to severe Aortic Stenosis
- Limit activity in high Dynamic Sports and high Static Sports
- No restriction needed for mild Aortic Stenosis
- Control Hypertension (40% of patients)
- Uncontrolled Hypertension in Aortic Stenosis is associated with increased mortality
- Start medications at low dose and titrate slowly, avoiding vasodilation and Syncope risk
- First-Line Medications
- Other Medications to use with caution or to avoid
- Manage comorbid Atrial Fibrillation (5% of patients) with rate control
- See Atrial Fibrillation Rate Control
- Use with caution Beta Blockers and Calcium Channel Blockers
- Risk of exacerbating Left Ventricular Systolic Dysfunction
- Reduce other Cardiovascular Risk
- See Cardiac Risk Management
- Tobacco Cessation
- Consider Aspirin prophylaxis
- Consider Statin for lipid lowering
- Statins do not reduce aortic valve calcification or stenosis
- However, Statins do reduce overall Cardiovascular Risk
- Use 10 year Cardiac Risk calculators (e.g. PREVENT) for indications and dosing
- Monitoring: Echocardiogram Frequency
- Mild Aortic Stenosis: Every 3-5 years
- Moderate Aortic Stenosis: Every 1-2 years
- Severe Aortic Stenosis: Every 6-12 months
XV. Management: Aortic Stenosis Staging-Based
- Stage A: Aortic Stenosis Risk Factors (but no current Aortic Stenosis)
- Risk factor reduction (see above)
- Stage B: Progressive Mild Aortic Stenosis (asymptomatic)
- Repeat Echocardiogram every 3-5 years
- No Exercise restriction
- Stage B: Progressive Moderate Aortic Stenosis (asymptomatic)
- Repeat Echocardiogram every 1-2 years
- Avoid strenuous Exercise (including high Dynamic Sports, high Static Sports)
- Stage C: Asymptomatic Severe Aortic Stenosis
- Cardiology referral
- Avoid strenuous Exercise or activity
- Repeat Echocardiogram every 6-12 months
- Aortic Valve Replacement indications (see below)
- Left Ventricular Ejection Fraction <50%
- Other cardiovascular surgery needed
- Maximum Aortic jet velocity (Vmax): >5 m/sec (or increases >0.3 m/sec in 1 year on serial echo)
- Positive Exercise Stress Test
- Stage D: Symptomatic Severe Aortic Stenosis (e.g. exertional Dyspnea, Syncope, Heart Failure)
- See below for acute management
- Prompt cardiology referral for aortic Valve Replacement
XVI. Management: Symptomatic and severe, Critical Aortic Stenosis (>40 mmHg across valve or aortic jet velocity >4.0 m/s)
- Admit symptomatic severe Aortic Stenosis and plan aortic Valve Replacement
- Consult Cardiothoracic surgery and interventional cardiology
- Consider valvuloplasty as a temporizing measure in Unstable Patients
- Maintain euvolemia with hydration
- Aortic Stenosis is a Preload dependent disorder
- Maintain normal Heart Rate
- Tachycardia and Bradycardia are poorly tolerated
- Approach: Hypertension
- See SCAPE management below
- Preferred agents
- ACE Inhibitors
- Amlodipine (Norvasc)
- Nitroprusside
- Consider in Critical Aortic Stenosis and ejection fraction <35%
- Khot (2003) N Engl J Med 348(18): 1756-63 [PubMed]
- Agents to use with caution
- Diuretics
- Indicated for Congestive Heart Failure with hypervolemia
- Use with caution (lowers LV filling pressure)
- Use Nitroglycerin only with caution
- Indicated for Congestive Heart Failure with hypervolemia
- Monitor Blood Pressure carefully
- Volume expansion may be required
- Use Beta Blockers with caution
- Indicated for rate control in Supraventricular Tachycardia
- Risk of Congestive Heart Failure
- Diuretics
- Agents to avoid
- Peripheral Alpha Adrenergic Antagonists
- Approach: Hypervolemia (CHF)
- Nitroglycerin and Diuretics may be used, but monitor closely for Hypotension
- In Critical Illness, may require ECMO or intraortic balloon bridging to aortic Valve Replacement
- Approach: Hypovolemia
- Hypotension is high risk in Aortic Stenosis
- MAP below 65 mmHg decreases coronary perfusion and decreases Cardiac Function
- May administer small fluid boluses in cycles with reassessment after each bolus
- HIgh risk for Fluid Overload (fine balance)
- Employ Vasopressors early
- Follow Point Of Care Cardiac Ultrasound (Cardiac Function, inferior vena cava)
- Vasopressors (Phenylephrine, Vasopressin, Norepinephrine)
- Administer at lowest effective dose for shortest period
- Vasopressors that do not effect Heart Rate are preferred (unless concurrent Bradycardia)
- Vasopressin and Phenylephrine constrict Afterload and improve Coronary Artery filling
- Start Vasopressin 0.04 units/min
- Add Phenylephrine or Norepinephrine as needed
- Alternatively, may increase Vasopressin to 0.06 units/min if used as single Vasopressor
- Hypotension is high risk in Aortic Stenosis
- Approach: Sympathetic Crashing Acute Pulmonary Edema (SCAPE)
- Fentanyl may suppress sympathetic overdrive
- Noninvasive Ventilation (CPAP)
- Consider vasodilators for Afterload reduction with caution
- Clevidapine
- Preferred for rapid on and off activity (contrast with longer acting Nicardipine)
- Able to be rapidly turned off in case of Hypotension
- Preferred for maintained Preload (contrast with venodilation with Nitroglycerin)
- Preferred for rapid on and off activity (contrast with longer acting Nicardipine)
- Clevidapine
- Approach: Advanced Airway
- Start with Noninvasive Ventilation (CPAP)
- Use Fentanyl as needed to facilitate patient comfort with CPAP
- May be sufficient Ventilatory management to avert Mechanical Ventilation
- Endotracheal Intubation
- Attempt Dissociative Awake Intubation or Awake Nasotracheal Intubation
- Use Ketamine for induction agent
- Avoid Paralytic Agent (loss of sympathetic drive and apnea)
- Start with Noninvasive Ventilation (CPAP)
- References
- Weingart and Swaminathan in Swadron (2022) EM:Rap 22(3): 2-4
XVII. Management: Aortic Valve Replacement
- Precautions
- Aortic valve area (AVA) <1 cm2 is criteria for stenosis unless completely normal cardiovascular testing
- In HFrEF, Stroke Volume is reduced, resulting in less severe Vmax (<4 m/sec) and mAVPG (<40 mmHg)
- However, aortic valve area will still be severely reduced at <1 cm2
- Known as Low Flare, Low Gradient Severe Aortic Stenosis
- Surgical evaluation should be prompt for severe Aortic Stenosis
- Do not Exercise Stress Test severe Aortic Stenosis with symptoms (high risk for adverse events)
- Consider stress test only if symptomatic status is unclear
- Severe Aortic Stenosis is a risk for sudden death
- Valve Replacement may be indicated even if ejection fraction low
- Valve Replacement is not effective if low ejection fraction and low valve gradient
- Carabello (2002) N Engl J Med 346:677-82 [PubMed]
- Do not Exercise Stress Test severe Aortic Stenosis with symptoms (high risk for adverse events)
- Indications for Aortic Valve Replacement
- Criteria 1: Severe Aortic Stenosis (see classification above) and
- Maximum Aortic jet velocity (Vmax): >4 m/sec
- Mean gradient: >40 mmHg
- Aortic valve area: <1 cm2
- Criteria 2: One of criteria below
- Symptomatic Aortic Stenosis
- Possible symptomatic Aortic Stenosis (esp. elderly) with abnormal stress test
- Symptoms or Hypotension
- Left Ventricular ejection fraction <50%
- Dobutamine Stress Echo with <=1 cm valve area or aortic jet velocity >=4 m/s
- Symptoms or Hypotension
- Heart Surgery (e.g. CABG) is already planned (consider AVR even if moderate Aortic Stenosis)
- Left ventricular ejection fraction <50%
- Severe aortic valve calcification or rapid progression (e.g. 0.3 m/s increase per year)
- Low-flow, low gradient severe Aortic Stenosis may initially be misdiagnosed as moderate Aortic Stenosis
- Most common in older women with Hypertension
- However Aortic valve area (AVA) will be<1 cm2
- Asymptomatic but near Critical Aortic Stenosis
- Aortic valve gradient >60 mmHg
- Aortic valve orifice <0.6 cm2
- Aortic jet velocity >5.0 m/s
- Nishimura (2005) Mayo Reviews Lecture, Rochester
- Criteria 1: Severe Aortic Stenosis (see classification above) and
- Aortic Valve Replacement Types
- Transcatheter Aortic Valve Replacement (TAVR)
- Catheter deployed collapsible metal mesh frame coated in cow or pig heart tissue
- Requires initial Warfarin (INR target 2-3) or DOAC for 3-6 months, then lifelong Aspirin 81 mg orally daily
- Consider with age >65 years or Life Expectancy <20 years (otherwise open surgical replacement is preferred)
- Efficacy and safety
- Averts the risks of major open thoracic surgery (Atrial Fibrillation, severe bleeding)
- Associated with higher vascular complications, Pacemaker requirement and reintervention requirement
- Similar short and longterm mortality as with standard open Valve Replacement
- Similar outcomes for bicuspid aortic valve as with standard open Valve Replacement
- Bioprosthetic Aortic Valve Replacement
- Mechanical Aortic Valve Replacement
- Open thoracic surgery with valves made of durable pyrolytic carbon/titanium wrapped in fabric (e.g. polyester)
- Requires lifelong Warfarin
- Target INR 2-3 if no additional risk factors indicating higher target range
- Target INR 2.5 to 3.5 Risk Factor Indications
- Atrial Fibrillation
- Known Hypercoagulable state OR history of prior Venous Thromboembolism
- Left Ventricular Systolic Dysfunction (HFrEF)
- Transcatheter Aortic Valve Replacement (TAVR)
- Post-Valve Replacement
- Anticoagulation (based on Aortic Valve Replacement Type)
- SBE Prophylaxis
XVIII. Complications
- Left Ventricular Hypertrophy
- Congestive Heart Failure
- Exacerbation of Coronary Artery Disease
- Sudden Death
XIX. Prognosis: Prior to Valve Replacement
- Mild Aortic Stenosis: Good (slow progression)
- Anticipate active and asymptomatic for 10-50 years
- Asymptomatic severe Aortic Stenosis
- At 5 years, 72% will die or have symptoms
- Recent data suggests sudden death rate is high
- Pellikka (2005) Circulation 111:3290-5 [PubMed]
- Symptomatic severe Aortic Stenosis: Poor prognosis
- Most patients will have symptom progression
- Anticipate death within 3 years in most patients
- Even mild pre-AVR symptoms predict a 2 year mortality >50%
XX. Prognosis: After Aortic Valve Replacement
- Consider transcatheter Valve Replacement (TAVR) in those who are at very high surgical risk
- Mortality at 30 days post-AVR: 3% (up to 4.5% if CABG performed at the same time)
XXI. Resources
- Late Aortic Stenosis (University of Washington School of Medicine)
XXII. References
- Kondos (1998) CMEA Medicine Review Lecture, San Diego
- Long and Gottlieb in Herbert (2022) EM:Rap 22(2): 13-5
- Schauer et al. (2016) Crit Dec Emerg Med 30(9):13-9
- Assi (1998) Postgrad Med 104(6):99-110 [PubMed]
- Bonow (1998) Circulation 98:1949-84 [PubMed]
- Bonow (2006) Circulation 114(5): e84-e231 [PubMed]
- Carabello (1997) N Engl J Med 337(1):32-41 [PubMed]
- Gottlieb (2018) J Emerg Med 55(1): 34-41 [PubMed]
- Grimard (2016) Am Fam Physician 93(5): 371-8 [PubMed]
- Grimard (2008) Am Fam Physician 78(6): 717-25 [PubMed]
- Lester (1998) Chest 113:1109-14 [PubMed]
- Otto (2006) J Am Coll Cardiol 47(11): 2141-51 [PubMed]
- Patel (2026) Am Fam Physician 114(2): 148-55 [PubMed]
- Shipton (2001) Am Fam Physician 63(11):2201-8 [PubMed]