Aortic stenosis

Highlights
Summary Overview

Basics
Definition Epidemiology Aetiology Pathophysiology

Prevention
Primary Secondary

Diagnosis
History & examination Tests Differential Step-by-step Criteria Guidelines Case history

Treatment
Details Step-by-step Guidelines Evidence

Follow Up
Recommendations Complications Prognosis

Resources
References Images Online resources Patient leaflets Credits Email Print Feedback Share Add to Portfolio Bookmark Add notes

History & exam

Key factors
presence of risk factors dyspnoea chest pain syncope

ejection systolic murmur S2 diminished and single carotid parvus et tardus

Other diagnostic factors

bleeding paradoxically split S2 Gallavardin's phenomenon

History & exam details

Diagnostic tests
1st tests to order
ECG echocardiogram including Doppler

Tests to consider
cardiac MRI cardiac catheterisation ECG exercise stress testing dobutamine stress echo Diagnostic tests details

Treatment details
Presumptive
clinically unstable medical therapy or balloon valvuloplasty

Acute
clinically stable: symptomatic o o o o o o surgical candidate: low risk surgical aortic valve replacement long-term anticoagulation long-term infective endocarditis antibiotic prophylaxis surgical candidate: high risk referral for surgical aortic valve replacement long-term anticoagulation long-term infective endocarditis antibiotic prophylaxis

o o o o o o o o

transcatheter valve replacement (TAVR) if available long-term anticoagulation long-term infective endocarditis antibiotic prophylaxis inoperable (surgical non-candidate) transcatheter valve replacement (TAVR) if available long-term anticoagulation long-term infective endocarditis antibiotic prophylaxis medical therapy balloon valvuloplasty clinically stable: asymptomatic

o o o

severe AS clinical and echo follow-up or referral for surgery non-severe AS: not undergoing bypass, valve, or aortic surgery clinical and echo follow-up non-severe AS: undergoing bypass, valve, or aortic surgery consideration of concomitant prophylactic valve replacement

Treatment details

Summary
Obstruction of blood flow across the aortic valve due to aortic calcification. Presentation includes shortness of breath with exertion, angina, or syncope. Characteristic murmur is systolic, mid-to-late peaking with a crescendo-decrescendo pattern, and radiates to the carotids. valve. Surgical aortic valve replacement has been the only effective therapy for aortic stenosis for over 50 years. However, with the advent of transcatheter valve therapies, patients and physicians have more options. Following valve replacement, patients are subject to the complications of prosthetic valves. Doppler echo is essential to the diagnosis and will show a pressure gradient across the stenotic aortic

Other related conditions

Assessment of dyspnoea Hypertrophic cardiomyopathy Cardiac arrest Chronic renal failure Deep vein thrombosis Infective endocarditis Assessment of syncope

Acute exacerbation of congestive heart failure Rheumatic fever Hypercholesterolaemia Chronic congestive heart failure

Definition
Aortic stenosis (AS) is the obstruction of blood flow across the aortic valve due to aortic calcification. It is a progressive disease that presents after a long subclinical period with symptoms of chest pain, syncope, and heart failure.

Epidemiology
AS is the most common valvular disease in the US and Europe and is the second most frequent cause for cardiac surgery. It is largely a disease of older people, and the prevalence increases with age. Patients typically present in the seventh or eighth decade of life. AS has been reported to be present in nearly 4% of patients 75 years, and in 2% to 7% of patients >65 years. [1] [2] It is preceded by aortic sclerosis (defined as aortic valve thickening without flow limitation), often suspected by the presence of an early-peaking, systolic ejection murmur, and confirmed by echocardiography. Nearly 25% of people 65 years have aortic sclerosis, and nearly 17% of people with aortic sclerosis will progress to AS in their lifetime. [3] The average time from diagnosis of aortic sclerosis to the development of moderate and severe AS is 6 and 8 years, respectively. [3] Patients with bicuspid valves and AS present nearly a decade earlier on average than patients with trileaflet valves. Congenital bicuspid aortic valves affect 0.9% to 1.36% of the general population with a 2:1 male:female predominance. [4] [5] Overall, about half of all aortic valve replacements are performed for congenitally malformed valves. A study of a large series of patients undergoing aortic valve replacement for AS found that in patients <50 years old, one third had unicuspid valves, and two-thirds had bicuspid valves. Among patients aged 50 to 70 years, two-thirds had bicuspid valves and one third had trileaflet valves. For patients >70 years old, 60% had trileaflet valves, and 40% had bicuspid valves. [6]

Aetiology
Calcification of normal trileaflet valves is the most common cause of AS in adults and accounts for as many as 80% of cases in the US and Europe. [2] Calcific aortic disease represents a spectrum ranging from aortic

sclerosis (defined as leaflet thickening without obstruction), to severe AS. Several risk factors have been associated with aortic sclerosis including smoking, hypertension, diabetes, LDL-cholesterol, and elevated C-reactive protein. Retrospective studies have shown that high LDL-cholesterol levels and smoking are associated with progression to AS, but causality has not been confirmed. [1] Congenitally bicuspid valves account for the majority of the remainder of cases. Patients with coarctation of the aorta and Turner's syndrome have a higher incidence of bicuspid valves. Rheumatic heart disease has historically been an important cause of AS, but due to improvements in treatment, it is now uncommon in industrialised countries. Rheumatic heart disease remains prevalent in developing nations. Other circumstances including connective tissue diseases, radiotherapy, and hyperlipoproteinaemia syndromes can cause AS, but these are unusual consequences of rare conditions. Chronic kidney disease is associated with abnormal calcium homeostasis, and it has been shown that AS progresses faster in patients with this condition.

Pathophysiology
Aortic calcification is no longer thought to reflect age-related wear and tear, and is recognised to be an active process. The valvular endocardium is damaged as the result of abnormal blood flow across the valve. Endocardial injury initiates an inflammatory process similar to atherosclerosis and ultimately leads to deposition of calcium on the valve. Calcification occurs slowly and is subclinical until the disease is fairly advanced. Progressive calcium deposition limits aortic leaflet mobility and eventually produces stenosis. Unicuspid and bicuspid valves experience abnormal shear and mechanical stresses from birth. Therefore, the pathological processes and resultant stenosis occur earlier than in trileaflet valves. [4] [7] [8] View image In rheumatic disease, an autoimmune inflammatory reaction is triggered by prior Streptococcusinfection that targets the valvular endothelium leading to inflammation and eventually calcification. AS imposes a pressure burden on the left ventricle, and ventricular muscle hypertrophies in response. This is an appropriate adaptive mechanism that

allows the ventricle to maintain a normal wall stress (afterload) despite the pressure overload produced by stenosis. As the stenosis worsens, the adaptive mechanism fails and left ventricular wall stress increases. Systolic function declines as wall stress increases, and eventually the heart fails. Many of the symptoms of AS can be attributed to LVH. The consequence of concentric LVH is a smaller, less compliant chamber. Thus, left ventricle enddiastolic pressure is increased, especially during periods of increased cardiac output (e.g., exercise), leading to the sensation of dyspnoea. Furthermore, hypertrophied heart muscle receives less blood than normal heart muscle and thus is subject to ischaemia that manifests as chest pain even in the absence of coronary atherosclerotic disease. Areas of relatively ischaemic heart muscle are potential sources of arrhythmia that may manifest as ventricular tachycardia and sudden cardiac death.

Primary prevention
There are no strategies regarding the primary prevention of AS; however, it remains to be seen if the incidence of AS will decrease with the increased recognition and treatment of cardiovascular disease risk factors that appear to contribute to aortic valve pathology.

Secondary prevention
Antibiotic prophylaxis for the prevention of infective endocarditis is no longer indicated in patients with AS, but prophylaxis is indicated in: [47]
People with prosthetic cardiac valves, or where prosthetic material has been used in valve repair People with previous infective endocarditis Specific patients with congenital heart disease Cardiac transplant recipients with valve regurgitation due to a structurally abnormal valve.

History & examination

Key diagnostic factorshide all
presence of risk factors (common)

Key factors include advanced age, congenitally bicuspid valve, rheumatic fever, and chronic kidney disease.

dyspnoea (common)

Shortness of breath on exertion is the most common presenting complaint. Dyspnoea occurs at a prevalence of 60%. [14]

chest pain (common)

Chest pain is frequently present and may be impossible to distinguish from pain due to CAD.

Chest pain occurs at a prevalence of 50%. [14] syncope (common)

Syncope is a classic symptom and may be caused by arrhythmia or postural hypotension related to AS.

The prevalence of syncope is 40%. [14] ejection systolic murmur (common)

A systolic murmur 3/6 is present with a crescendo-decrescendo pattern that peaks in mid-systole and radiates to the carotid.View image

The murmur is loudest at the right upper sternal border. S2 diminished and single (common)

With progression of AS, the aortic component of the second heart sound becomes soft or absent due to decreased mobility of the aortic valve leaflets.

Aortic valve closure is delayed and often coincides with pulmonic valve closure, producing a single second heart sound.

carotid parvus et tardus (uncommon)

The carotid upstroke is frequently delayed and diminished in severe AS (parvus et tardus). This finding is often absent in older patients with less compliant vasculature. In these

circumstances, palpation of the brachial artery may reveal this finding. Other diagnostic factorshide all bleeding (uncommon)

Patients frequently complain of epistaxis or bruising. Turbulent flow across the stenotic valve leads to an acquired von Willebrand deficiency.

The prevalence of bleeding is 20%. [5] paradoxically split S2 (uncommon)

With more severe stenosis, aortic valve closure may become so delayed that it follows pulmonic valve closure during expiration, producing the paradoxically split S2.

May be accentuated by left bundle branch block. Gallavardin's phenomenon (uncommon)

A musical-quality, holosytolic murmur is present at the apex of the heart that occurs in older patients with calcific AS, which may mimic mitral regurgitation. factorshide all

Risk

Strong age >60 years

Aortic stenosis (AS) typically presents in the seventh or eighth decade for patients with trileaflet valves and 5 to 10 years earlier for patients with bicuspid valves. [5]

congenitally bicuspid aortic valve

Accounts for about 20% of cases. Among patients who undergo aortic valve replacement, patients with bicuspid valves are more commonly male while patients with tricuspid valves are more commonly female. [5]

Unicuspid and bicuspid valves experience abnormal shear and mechanical stresses from birth. Therefore, the pathological processes and resultant stenosis occur earlier than in trileaflet valves. [4] [7] [8] View image

rheumatic heart disease

Common cause of AS in developing countries. In rheumatic disease, an autoimmune inflammatory reaction is triggered by prior Streptococcus infection that targets the valvular endothelium leading to inflammation and eventually calcification.

chronic kidney disease

Aortic valve calcification occurs in up to 55% of dialysis patients >65 years of age. [9] Dialysis patients also have more rapid annual decrease in valve area (0.23 cm^2 versus 0.1 cm^2) compared with patients with normal renal function. [9]

Weak radiotherapy

AS is a rare complication of mediastinal radiotherapy that presents at least 2 decades following treatment.[10]

high LDL cholesterol

Retrospective studies have demonstrated that high LDL-cholesterol levels are associated with AS, and several small studies have found that statin therapy targeting LDL-cholesterol slows the haemodynamic progression of AS. [11] However, these findings were not verified in a large prospective study. [12]

hyperlipoproteinaemia

Familial hypercholesterolaemia is a rare genetic disorder that can contribute to early-onset calcification of the aortic valve and the aortic root. [13]

Diagnostic tests
1st tests to orderhide all
Test

ECG overload. [14] View image heart disease.

The ECG is abnormal in >90% of patients with AS, with the most common abnormality being LVH due to pressur

Evidence of LVH and absent Q waves helps distinguish AS from other conditions such as aortic sclerosis with isc

Patients with AS can often have conduction disease manifesting as AV block, hemiblock, or bundle branch block echocardiogram including Doppler test are high. [18]View image

Doppler echo is the best test for the initial diagnosis and subsequent evaluation of AS. The sensitivity and specif

Tests to considerhide all

Test

cardiac MRI

Cardiac MRI (cMRI) provides detailed, dynamic images of the heart. It allows for analysis of cardiac function and subvalvular stenosis related to a subvalvular membrane.

haemodynamics. Furthermore, cMRI is especially useful when trying to distinguish between true valvular stenosi

Due to the cost and complexity of cMRI, transthoracic echocardiogaphy remains the preferred test for evaluating cMRI is a good option when echocardiography fails to yield quality images. cardiac catheterisation Catheterisation allows for direct measurement of the pressure gradient. The sensitivity and specificity of the test high.

Due to the invasive nature, this test is useful for diagnosis only when the echocardiogram is inconclusive or discr from other findings. ECG exercise stress testing Consider in asymptomatic patients and those with equivocal symptoms.

Severe AS has been considered by some experts to be an absolute contraindication to exercise testing due to th

perceived risk of harm. However, many experts advocate the use of symptom-limited exercise testing in asympto [B Evidence] Exercise echocardiography may add additional diagnostic and prognostic information. [19] [20] Symptomatic patients should not have exercise testing, and should be referred for surgery. dobutamine stress echo ejection fraction), in order to identify pseudostenosis and the presence of contractile reserve.

patients with severe AS as a means of risk-stratification for the development of symptoms, and for the need for s

Useful for the population of patients with a low transvalvular gradient and left ventricular systolic dysfunction (low

Patients with pseudostenosis do not have severe AS, and should not be referred for surgical valve replacement.

The presence of contractile reserve suggests a better prognosis and lower surgical risk with surgical valve replac

Differential diagnosis
Condition Aortic sclerosis Differentiating signs/symptoms Differentiating tests

The murmur of aortic sclerosis is typically less intense and non-radiating, and the S2 is normal and physiologicall y split. The carotid upstroke is not delayed.

Transthoracic Doppler echo will demonstrate no significant pressur across the aortic valve with aortic sclerosis.

Signs and symptoms of heart failure or chest pain should prompt an echocardiogr am but cannot by themselves reliably distinguish the 2 conditions.

Ischaemic heart disease

AS and CAD frequently coexist and it is

In ischaemic heart disease, common ECG findings include Q wave thickening or wall motion abnormalities.

and the echocardiogram often shows segments of the myocardium

difficult to determine their relative contributions to cardiac complaints when both are present in the same patient.

In AS, Q waves are absent in the ECG; regional wall motion abnorm

the echocardiogram; and coronary arteries are normal upon cardiac

In practice, for patients with severe AS and concomitant CAD, both are treated at the time of surgery.

Hypertrophic cardiomyopathy (HCM)

HCM is a heterogeneou s disease that can produce subvalvular stenosis and a murmur that is identical to AS.

Typical echocardiography findings include asymmetric hypertrophy most prominently affected. Dynamic obstruction is observed at the

calcification of the valve is often absent, especially in younger patie

Hand gripping and squatting manoeuvres are helpful in distinguishing HCM from AS. These manoeuvres

do not greatly affect the intensity of the murmur of AS; however, hand gripping, which increases afterload, will soften the HCM murmur. Standing up after squatting will decrease cardiac return and ventricular preload, which increases the murmur of HCM.

Step-by-step diagnostic approach

Clinical evaluation
Many cases of AS are diagnosed during the subclinical phase while a murmur, noted on physical examination, is being investigated. Even patients with severe AS may be truly asymptomatic. A careful history is important to determine if the patient has altered his or her habits in response to slowly worsening stenosis. Complaints of decreased exercise tolerance, shortness of breath on exertion, chest pain, syncope, or near syncope, and heart failure symptoms should prompt consideration of AS. Patients frequently complain of epistaxis or

bruising. This may be due to turbulent flow across the stenotic valve leading to an acquired von Willebrand deficiency. The physical examination is the most important screening tool for valvular heart disease. A complete cardiac examination including precordial palpation, auscultation with attention to murmurs and the aortic closure sound, and evaluation of arterial and venous pulsation is essential in generating clinical suspicion for AS. Murmurs are generally graded on a scale of 1 to 6
Grade 1: murmur is faint and heard only with effort Grade 2: murmur is faint but easily detected Grade 3: murmur is loud Grade 4: murmur is very loud and associated with a palpable thrill Grade 5: murmur is so loud that it can be heard with only the edge of the stethoscope Grade 6: murmur is extremely loud and heard even when the stethoscope is no longer in contact with the patient.

The typical murmur of AS is a systolic murmur 3/6, with a diamond-shaped crescendo-decrescendo pattern that peaks in mid-systole and radiates to the carotid arteries.View image The murmur is generally loudest at the right upper sternal border and terminates with S2 and the end of systole. As the severity of stenosis worsens, the murmur peaks later in systole and may be accompanied by a palpable thrill. The aortic heart sound (A2) is delayed as the systolic ejection period becomes prolonged. In severe stenosis, paradoxical splitting of the second heart sound may be noted such that in expiration, the pulmonic sound (P2) is heard before A2. The intensity of the second heart sound tends to be diminished as the severity of stenosis increases and leaflet mobility is reduced. Uncommon findings include the presence of a holosystolic murmur at the apex (Gallavardin's phenomenon), mimicking the murmur of mitral regurgitation. A delayed and diminished carotid upstroke (carotid parvus et tardus) may occur with severe AS, although this finding is often difficult to distinguish in

elderly patients. In these circumstances, palpation of the brachial artery may reveal this finding.

ECG
An ECG is indicated in the initial work-up of all patients and is abnormal in >90% of patients with AS, with the most common abnormality being LVH due to pressure overload. [14] View imageEvidence of LVH and absent Q waves helps distinguish AS from other conditions such as aortic sclerosis with ischaemic heart disease. Patients with AS often have conduction disease manifesting as AV block, hemiblock, or bundle branch block.

Doppler echo
Echocardiography is the test of choice in the evaluation of suspected AS and for the evaluation of murmurs detected on physical examination. The American College of Cardiology/American Heart Association recommends that asymptomatic patients with a grade 3 or louder mid-peaking systolic murmur and symptomatic patients with a grade 2 systolic mid-peaking murmur be referred to echocardiography. [5] Although murmur intensity does not correlate well with the haemodynamic significance of the associated lesion, grade 3 murmurs are generally thought to reflect more significant lesions and thus warrant further evaluation with an echocardiogram. In practice most patients with suspected cardiac disease and a murmur on examination should have an echocardiogram.View image Transthoracic Doppler echo can reliably and accurately detect the presence of a pressure gradient across the aortic valve. It can also assess left ventricle function and the presence of hypertrophy. It is essential for the diagnosis of AS and for serial evaluation once the diagnosis has been established. Measurements taken during echo examination are used to grade the severity of AS. [5]

Cardiac MRI
Cardiac MRI (cMRI) provides detailed, dynamic images of the heart. It allows for analysis of cardiac function and haemodynamics. Furthermore, cMRI is especially useful when trying to distinguish between true valvular stenosis and subvalvular stenosis related to a subvalvular membrane. Due to the cost and complexity of cMRI, transthoracic echocardiogaphy remains the preferred test for evaluating AS, but cMRI is a good option when echocardiography fails to yield quality images.

ECG exercise stress testing

Symptomatic patients should not have exercise testing and should be referred for surgery. For those without symptoms, exercise testing may provide clinically important information. Nearly 40% of patients with asymptomatic AS will develop symptoms with exercise testing. [15] In a group of patients with asymptomatic severe AS, a positive exercise stress test (defined as the onset of symptoms, ST changes, abnormal BP response, or complex ventricular arrhythmia) was highly predictive of the onset of symptoms or the need for surgery. At 24 months, only 19% with a positive exercise test remained symptom-free or without valve replacement compared with 85% of those with a negative test. [16] Subsequent work found that the development of symptoms on exercise testing was the strongest predictor for the onset of spontaneous symptoms, especially among patients <70 years old in whom symptoms of fatigue and breathlessness are more specific than in more elderly patients. [17] Severe AS has been considered by some experts to be an absolute contraindication to exercise testing due to the perceived risk of harm. However, many experts advocate the use of symptom-limited exercise testing in asymptomatic patients with severe AS as a means of risk-stratification for the development of symptoms, and for the need for surgery.[B Evidence]However, symptomatic patients should not have exercise testing and should be referred for surgery.

Dobutamine stress echo

This test is useful for patients with a low transvalvular gradient and left ventricular systolic dysfunction (low ejection fraction), in order to identify pseudostenosis and the presence of contractile reserve. Patients with pseudostenosis do not have severe AS, and should not be referred for surgical valve replacement. The presence of contractile reserve suggests a better prognosis and lower surgical risk with surgical valve replacement.

Cardiac catheterisation
Cardiac catheterisation is no longer the test of choice for diagnosis of AS. As a diagnostic tool it is used only when echo-Doppler examination is inconclusive, or there is a discrepancy between non-invasive findings and the physical examination. Catheterisation remains essential, however, prior to aortic valve replacement for the evaluation of coronary artery anatomy.

Click to view diagnostic guideline references.

Diagnostic criteria

Grading severity of AS [5]

Mild:
Jet velocity <3.0 m/second Mean pressure gradient <25 mmHg Valve area >1.5 cm^2.

Moderate:
Jet velocity 3.0 to 4.0 m/second Mean pressure gradient 25 to 40 mmHg Valve area 1.0 to 1.5 cm^2.

Severe:
Jet velocity >4.0 m/second Mean pressure gradient >40 mmHg Valve area <1.0 cm^2.

Case history
A 78-year-old man presents to his primary care physician complaining of 2 months of progressive shortness of breath on exertion. He first recognises having to catch his breath while gardening and is now unable to walk up the stairs in his house without stopping. Previously he was healthy and active without similar complaints. On physical examination there is a loud systolic murmur at the right upper sternal border radiating to the carotid vessels.

Other presentations
AS is a progressive disease that presents after a long subclinical period with symptoms of chest pain, syncope, and heart failure. While the most common complaint is dyspnoea with exertion, patients also frequently note syncope or chest pain that may be identical to that caused by CAD. Many cases of AS are diagnosed during the subclinical phase while a murmur noted on physical

examination is being investigated. Even with severe AS, patients may be truly asymptomatic. A careful history is important to determine if the patient has altered his/her habits in response to slowly worsening stenosis.

Treatment Options
Treatment Patient group clinically unstable line 1st Treatmenthide all

medical therapy or balloon valvuloplasty

All patients need to be stablised prior to surgery or transcatheter valve replacement. There has been concern that vasodilator therapy could be potentially dangerous, due to the possibility that a stenotic valve might permit only a fixed cardiac output, leading to hypotension. However, more recent studies have suggested that this is not accurate.[C Evidence] Vasodilator and beta-blocker therapy is commonly not given pre-operatively on the day of surgery.

Sometimes, balloon valvuloplasty is considered as a temporising measure prior to surgery or transcatheter valve replacement. Balloon valvuloplasy is a percutaneous procedure, done in the cardiac catheterisation laboratory, in which a balloon is forcefully inflated across the aortic valve to relieve stenosis.View image

Presumptive
Treatment Patient group clinically stable: symptomatic surgical candidate: low risk 1st line Treatmenthide all

surgical aortic valve replacement

The Society of Thoracic Surgery (STS) score [Society of Thoracic Surgeons: Risk calculator] (external link) and the logistic European System for Cardiac Operative Risk Evaluation (EuroSCORE) [23] [24] [25] are 2 risk assessment

Treatment Patient group clinically stable: symptomatic line Treatmenthide all

tools, which are helpful as part of a global estimation of an individual patient's operative risk.

Surgical aortic valve replacement is the only therapy with confirmed survival benefit. The accumulated experience and acceptable operative risk profile make surgical valve replacement the appropriate therapy for most symptomatic patients with AS.

Prosthetic aortic valves used in surgical valve replacement may be mechanical or bioprosthetic.

adjunct [?]

long-term anticoagulation

Indicated in those patients who have had aortic valve replacement using prosthetic mechanical valves. Not required if bioprosthetic valves are used. Antibiotic prophylaxis for the prevention of infective endocarditis is no longer indicated in patients with AS, but prophylaxis is indicated in people with prosthetic cardiac valves, or where prosthetic material has been used in valve repair. [47]

plus [?]

long-term infective endocarditis antibiotic prophylaxis

surgical candidate: high risk

1st

referral for surgical aortic valve replacement

It is important all patients are referred for surgical assessment, even if they are initially considered high surgical risk. A decision not to operate usually has to be agreed by 2 separate surgeons.

The Society of Thoracic Surgery (STS) score [Society of Thoracic Surgeons: Risk calculator] (external link) and the logistic European System for Cardiac Operative Risk Evaluation (EuroSCORE) [23] [24] [25] are 2 risk assessment tools, which are helpful as part of a global estimation of an individual patient's operative risk.

Older patients and patients with low-flow, lowgradient AS are at a higher operative risk. [48] [49] [50] [51] [52] [53] [54]

Treatment Patient group clinically stable: symptomatic line Treatmenthide all

However, valve replacement results in significant survival benefit and symptomatic improvement for most, so referral for assessment is important.

The dobutamine stress test may be used to identify those patients with low-flow low-gradient AS with the capability to improve ejection fraction and stroke volumes. These patients do better with surgery than those who lack adequate contractile reserve.

adjunct [?]

long-term anticoagulation

Indicated in those patients who have had aortic valve replacement using prosthetic mechanical valves. Not required if bioprosthetic valves are used. Antibiotic prophylaxis for the prevention of infective endocarditis is no longer indicated in patients with AS, but prophylaxis is indicated in people with prosthetic cardiac valves, or where prosthetic material has been used in valve repair. [47]

plus [?]

long-term infective endocarditis antibiotic prophylaxis

2nd

transcatheter valve replacement (TAVR) if available

It is important all patients are referred for surgical assessment, even if they are initially considered high surgical risk.

The Society of Thoracic Surgery (STS) score [Society of Thoracic Surgeons: Risk calculator] (external link) and the logistic European System for Cardiac Operative Risk Evaluation (EuroSCORE) [23] [24] [25] are 2 risk assessment tools, which are helpful as part of a global estimation of an individual patient's operative risk.

During TAVR, a new valve is mounted on a stent and deployed through a catheter, entering the heart either via the femoral artery, or through the cardiac apex after a minimally invasive thoracotomy. Transaxillary and the transaortic routes may also be used.

Treatment Patient group clinically stable: symptomatic line Treatmenthide all

Advantages include the avoidance of cardiopulmonary bypass and median sternotomy.

Patients at high risk from surgery may be offered surgery or TAVR. In the PARTNER study comparing surgery and TAVR in high-risk patients, mortality and symptom reduction were similar at 2 years for each modality. [33] Peri-procedural risks varied at 30 days; vascular complications and neurologic events occurred more frequently after TAVR, while major bleeding and new-onset atrial fibrillation more commonly followed surgery. [34] At 2 years, echocardiographic improvements in valve area and mean gradients were similar in both groups, but paravalvular aortic regurgitation was encountered more frequently after TAVR. [33]

It must be emphasised that despite the encouraging results, TAVR is a relatively new technology, and it is unclear at this time how the durability of transcatheter valves will compare with surgical prostheses.

adjunct [?]

long-term anticoagulation

Indicated in those patients who have had aortic valve replacement using prosthetic mechanical valves. Not required if bioprosthetic valves are used. Antibiotic prophylaxis for the prevention of infective endocarditis is no longer indicated in patients with AS, but prophylaxis is indicated in people with prosthetic cardiac valves, or where prosthetic material has been used in valve repair. [47]

plus [?]

long-term infective endocarditis antibiotic prophylaxis

inoperable (surgical noncandidate)

1st

transcatheter valve replacement (TAVR) if available

Patients may be considered not a surgical candidate for various reasons (e.g., they may be stable but the risks of surgery are considered too great).

For inoperable patients (i.e., surgical non-

Treatment Patient group clinically stable: symptomatic line Treatmenthide all

candidates), TAVR is the preferred treatment modality. The PARTNER trial comparing standard therapy, including balloon aortic valvuloplasty, with TAVR in patients deemed inoperable, found a 20% absolute reduction in mortality at 1 year in favour of TAVR. [36] These differences in survival were sustained at 2 years. [37]

During TAVR, a new valve is mounted on a stent and deployed through a catheter, entering the heart either via the femoral artery, or through the cardiac apex after a minimally invasive thoracotomy. Transaxillary and the transaortic routes may also be used. Advantages include the avoidance of cardiopulmonary bypass and median sternotomy.

adjunct [?]

long-term anticoagulation

Indicated in those patients who have had aortic valve replacement using prosthetic mechanical valves. Not required if bioprosthetic valves are used. Antibiotic prophylaxis for the prevention of infective endocarditis is no longer indicated in patients with AS, but prophylaxis is indicated in people with prosthetic cardiac valves, or where prosthetic material has been used in valve repair. [47]

plus [?]

long-term infective endocarditis antibiotic prophylaxis

2nd

medical therapy

The advent of transcatheter valve replacement (TAVR) has made it possible to relieve AS in the majority of patients, regardless of their physical condition. However, just because valve replacement may be done, does not mean that it should be done. The decision to replace the aortic valve, either surgically or via catheter, should consider the expected benefit. Patients with terminal illness, significant dementia, or advanced comorbidities in

Treatment Patient group clinically stable: symptomatic line Treatmenthide all

whom valve replacement would not be expected to provide a meaningful improvement in life should not be referred for valve replacement.

There is no medical treatment that has been shown to improve survival in patients with aortic stenosis. While the role of statin therapy is established for atherosclerotic disease prevention, randomised trials of statins in patients with AS have not found improvements in AS progression. [40] [12] [41]Medical therapy should thus target comorbid conditions such as CAD, hyperlipidaemia, hypertension, and heart failure.

AS patients with heart failure symptoms are frequently treated with vasodilators such as ACE inhibitors and diuretics, [42] though caution is required to avoid complications such as hypotension and syncope. In critically ill patients with AS and left ventricular dysfunction, sodium nitroprosside has been used to improve haemodynamic parameters in the acute setting; additional data are limited. [43]

2nd

balloon valvuloplasty

A reasonable palliative option for patients who are highly symptomatic yet too high-risk to undergo operative repair, and where transcatheter valve replacement is not available or contraindicated.

A percutaneous procedure, done in the cardiac catheterisation laboratory, in which a balloon is forcefully inflated across the aortic valve to relieve stenosis.View image

Re-stenosis rates are high at 6 months and there is no confirmed improvement in mortality. Patients do have symptomatic relief, and there is some evidence that serial dilations may improve survival. [39]

Treatment Patient group clinically stable: symptomatic line Treatmenthide all

May occasionally be used as a bridge to more definitive therapy (e.g., aortic valve replacement surgery) if this is deemed to be possible at a later date.

clinically stable: asymptomatic severe AS 1st

clinical and echo follow-up or referral for surgery

Studies have reported significant differences in survival between those with preoperative ejection fraction (EF) >50% and patients with EF <50%. [44]Delaying surgery in these patients may lead to irreversible LV dysfunction and worsened survival.

The American College of Cardiology/American Heart Association (ACC/AHA) guidelines recommend that if coronary artery bypass, aortic, and non-aortic valve surgery is due to be performed, that severely stenotic valves ought to be replaced concomitantly. [5] [22]

Exercise testing may provide clinically important information in asymptomatic patients with severe AS and normal LV systolic function. It is recommended as a means of identifying those who are likely to develop symptoms or may benefit from earlier surgical referral. Valve replacement in asymptomatic patients with an abnormal exercise response receives a class IIB recommendation in the 2006 ACC/AHA guidelines. [5] [22]

When surgery is not performed, patients with severe AS should have an echocardiogram annually in addition to regular clinical follow-up. [5] [22]

non-severe AS: not undergoing bypass, valve, or aortic surgery

1st

clinical and echo follow-up

Serial transthoracic echocardiograms are recommended every 3 to 5 years in asymptomatic patients with mild stenosis, and every 1 to 2 years in

Treatment Patient group clinically stable: symptomatic line Treatmenthide all

asymptomatic people with moderate stenosis.

Patients also require clinical follow-up regularly (frequency less well defined).

non-severe AS: undergoing bypass, valve, or aortic surgery

1st

consideration of concomitant prophylactic valve replacement

When patients with mild or moderately stenotic valves undergo cardiac surgery, the decision to replace the valve is more difficult compared with the decision when there is severe stenosis. It balances the increased risk of adding aortic valve replacement to the planned surgery with the future likelihood of AS progressing to a severe, symptomatic state.

The American College of Cardiology/American Heart Association (ACC/AHA) guidelines give a class IIB endorsement for aortic valve replacement in patients with asymptomatic moderate AS undergoing bypass, valve, or aortic surgery. [5] [22]

Acute

Treatment approach
Various patient factors will influence the type of therapy recommended, including:
The presence or absence of symptoms Cardiac function and severity of stenosis Suitability for surgery and assessment of surgical risk.

Treatment of AS is primarily surgical, and is reserved for patients with symptoms or left ventricular systolic dysfunction. Symptom onset is a significant milestone and portends a poor prognosis, with an average survival of only 2 to 3 years without surgery. Advances in prosthetic-valve design, cardiopulmonary bypass, surgical technique, and anaesthesia have steadily improved outcomes for aortic valve surgery. [5] [22] Surgical aortic valve replacement has been the only effective therapy for AS for over 50 years.

However, with the recent advent of transcatheter valve therapies, patients and physicians have more options.

Clinical decisions: assessment of surgical risk

The decision to refer patients for surgery or transcatheter aortic valve replacement (TAVR) depends on their estimated surgical risk. Several surgical risk models have been developed, but the logistic EuroScore [23] [24] [25] and Society of Thoracic Surgery risk model [Society of Thoracic Surgeons: Risk calculator] (external link) are the most widely used. These risk models help the physician to categorise each patient as follows:
Low risk for surgery High risk for surgery Inoperable.

Patients at low risk for mortality or major morbid events are referred for valve replacement surgery. In high-risk patients, physicians should discuss the availability and relative merits of transcatheter aortic valve replacement (TAVR); these patients may be offered TAVR or valve replacement surgery. Inoperable patients (i.e., surgical non-candidates), such as those with prohibitively high surgical risk, advanced lung disease, chest wall deformity, or extensive aortic calcification, should be referred for TAVR if they are deemed to have the potential to benefit. [26]

Treatment decisions in elderly and frail patients

The advent of TAVR has made it possible to relieve AS in the majority of patients, regardless of their physical condition. However, just because valve replacement may be done, does not mean that it should be done. The decision to replace the aortic valve, either surgically or via catheter, should consider the expected benefit. Patients with terminal illness, significant dementia, or advanced comorbidities in whom valve replacement would not be expected to provide a meaningful improvement in life should not be referred for valve replacement.

Surgical aortic valve replacement

Surgery is the standard of care for symptomatic, severe AS. Over 24,000 isolated aortic valve replacements are performed in the US annually, making

AS the most common indication for valve surgery. The overall mortality for isolated surgical aortic valve replacement is reported to be 3.2%. [27] Studies have found that there is a near-normalisation of life expectancy following surgery and significant improvement in quality of life for the vast majority of patients. Prosthetic aortic valves used in surgical valve replacement may be mechanical or bioprosthetic. The type of prosthesis used depends on patient preference, but there has been trend towards greater utilisation of bioprosthetic valves in recent years. [27] Each type has relative merits and drawbacks.
o Mechanical valves: Less prone to degeneration and subsequent re-operation for valve failure compared with bioprosthetic valves o o Patients require subsequent systemic anticoagulation to prevent valve thrombosis. Bioprosthetic valves: More prone to degeneration and subsequent re-operation for valve failure compared with mechanical valves o Patients do not need systemic anticoagulation.

Outcome data comparing bioprosthetic and mechanical valves are difficult to interpret because bioprosthetic valve technology has improved significantly since the time of the largest randomised trials. Studies using first-generation bioprosthetic valves found a trend towards improved survival with mechanical valves. This became apparent after a decade post-surgery when re-operation for degenerative bioprosthetic valves began to occur. Outcomes were particularly bad among patients <65 years receiving bioprosthetic valves, suggesting that patients <65 years with an otherwise normal life expectancy should receive mechanical prosthesis. [28] [29] In contrast, newer studies have found no differences in mortality after correcting for preoperative severity of heart disease, regardless of age. They do note that bioprosthetic valves are more likely to require re-operation for valve failure. [30] [31] Overall, there is a trend among surgeons towards greater use of bioprosthetic valves. From 1997 to 2006, the usage of bioprosthetic valves in North America increased from 44% to 78.4%. [27] This trend reflects greater confidence in the durability of second- and thirdgeneration bioprosthetic valves.

Choices regarding types of aortic valve prosthesis must balance the risk of bleeding due to the need for systemic anticoagulation with mechanical valves, versus the increased possibility of re-operation with bioprosthetic valves. Physicians should discuss the merits and drawbacks of each type of valve, but ultimately the decision is up to the patient.

Transcatheter aortic valve replacement (TAVR)

Since the initial TAVR in 2002, over 50,000 procedures have been performed worldwide. [32]The procedure builds on the current techniques employed in the cardiac catheterisation laboratory for coronary angiography and intervention. Unlike surgical valve replacement, TAVR is done on the beating heart without the need for a sternotomy or cardiopulmonary bypass. Instead, catheters are introduced into the heart via several potential arterial access points, and a stent-mounted prosthetic valve is placed within the native aorta. Several different transcatheter heart valves exist, each with a unique delivery system and technique, but the basic premise remains the same. The advantages of this minimally invasive approach include the avoidance of cardiopulmonary bypass and median sternotomy. These benefits have allowed invasive cardiologists and cardiothoracic surgeons to offer aortic valve replacement to sicker and more complex patients. Patients at high risk from surgery may be offered surgery or TAVR. In the US PARTNER study comparing high-risk surgery and TAVR in high-risk patients, mortality and symptom reduction were similar at 2 years for each modality.[A Evidence] [33] Peri-procedural risks varied at 30 days; vascular complications and neurologic events occurred more frequently after TAVR, while major bleeding and new-onset atrial fibrillation more commonly followed surgery. [34] Scores on health-related quality-of-life assessments improved more rapidly with TAVR but were similar at 12 months. [35] At 2 years, echocardiographic improvements in valve area and mean gradients were similar in both groups, but paravalvular aortic regurgitation was encountered more frequently after TAVR. [33] It must be emphasised that, despite the encouraging results, TAVR is a relatively new technology and it is unclear at this time how the durability of transcatheter valves will compare with surgical prostheses. For patients deemed inoperable (i.e., surgical non-candidates), TAVR is the preferred treatment modality. [26] The PARTNER trial comparing standard therapy, including balloon aortic valvuloplasty, with TAVR in inoperable patients demonstrated a 20% absolute reduction in mortality at 1 year in favour of TAVR. [36] These differences in survival were sustained at 2 years. [37] Heart failure symptoms improved between 30 days and 6 months,

and were stable at 2 years, such that 83% of surviving TAVR patients were in New York Heart Association Class I/II, compared with only 8% at baseline. [37] [36] Patients treated with TAVR also showed significant improvements on assessments of health-related quality of life when compared to those receiving standard therapy. [38]

Balloon aortic valvuloplasty

For patients who are acutely symptomatic, or in cardiogenic shock, balloon valvuloplasty is a reasonable option as a bridge to surgery or TAVR. This is a percutaneous procedure done in the cardiac catheterisation lab in which a balloon is forcefully inflated across the aortic valve to relieve stenosis.View image Unfortunately, re-stenosis rates are high at 6 months and there is no shown improvement in mortality following valvuloplasty. However, patients do generally experience improvement in haemodynamics and symptoms, which may provide an opportunity for more definitive care. [39]

Medical therapy
There is no medical treatment that has been shown to improve survival in patients with aortic stenosis. While the role of statin therapy is established for atherosclerotic disease prevention, randomised trials of statins in patients with AS have not found improvements in AS progression. [40] [12] [41] Medical therapy should thus target comorbid conditions such as CAD, hyperlipidaemia, hypertension, and heart failure. AS patients with heart failure symptoms are frequently treated with vasodilators such as ACE inhibitors and diuretics, [42] though caution is required to avoid complications such as hypotension and syncope. In critically ill patients with AS and left ventricular dysfunction, sodium nitroprosside has been used to improve haemodynamic parameters in the acute setting; additional data are limited. [43]

Asymptomatic AS
Severe AS
Surgical referral is standard practice for patients with severe AS but without symptoms, depending on the circumstances. This is particularly important in patients with ejection fraction (EF) measurements of <50%. [5] [22] Studies have reported significant differences in survival (beginning as early as 3 years post-valve replacement) between those with preoperative EF >50% and patients with EF <50%. [44] Delaying surgery in these patients may lead to irreversible LV dysfunction and worsened survival.

In the absence of symptoms or left ventricular dysfunction, there are scenarios in which a severely stenotic valve should be replaced at the same time as other surgery is performed (e.g., where patients also require coronary artery bypass, aortic, or non-aortic valve surgery).[5] [22]

There is a sizable population of patients with severe AS who are asymptomatic with normal LV systolic function, and not requiring other types of cardiac surgery. The first determination to be made in these patients is that they are truly without symptoms, by a thorough history focused on activity level and changes in functional capacity. For those without symptoms, exercise testing may provide clinically important information. Valve replacement in asymptomatic patients with an abnormal exercise response is recommended in the 2006 American College of Cardiology/American Heart Association (ACC/AHA) guidelines.

It is recommended that patients with severe AS have an echocardiogram annually. [5] [22]

Mild or moderate AS
When patients with mild or moderately stenotic valves undergo cardiac surgery, the decision to replace the valve is more difficult and balances the increased risk of adding aortic valve replacement to the planned surgery with the future likelihood of AS progressing to a severe, symptomatic state. Prophylactic valve replacement at the time of coronary bypass surgery has been studied, though no large prospective, randomised controlled trials exist. A review of outcomes from 1995 to 2000 of the Society of Thoracic Surgery (STS) database found that if patients had a peak aortic gradient >30 mmHg and were <70 years (correlating with a more moderate degree of stenosis), they benefited from prophylactic valve replacement at the time of coronary artery bypass surgery. [45] These conclusions were supported by a subsequent retrospective analysis that found a significant survival advantage at 8 years in favour of prophylactic valve replacement at the time of bypass surgery for those with moderate but not mild AS. [46] The American College of Cardiology/American Heart Association (ACC/AHA) guidelines recommend that aortic valve replacement is reasonable for patients with asymptomatic moderate AS undergoing bypass, valve, or aortic surgery. [5] [22] Serial transthoracic echocardiograms in asymptomatic patients with mild stenosis is recommended every 3 to 5 years. [5] [22] People with moderate stenosis are recommended to have transthoracic echocardiograms every 1 to 2 years. [5] [22] These patients also require clinical follow-up regularly (frequency of visits less well defined).

Monitoring
For asymptomatic patients, close follow-up is imperative. While symptoms generally do not occur unless the patient has severe AS, patients with moderate AS need to be questioned thoroughly about symptoms, as the patient may often attribute functional decline to other causes such as age. A large prospective study of asymptomatic patients with severe AS showed that at 5 years only 20% were free of cardiovascular death or aortic valve replacement (AVR). [60] It may be appropriate to perform an exercise test in asymptomatic patients with severe AS to assess symptoms and physiological

response to exercise. Those who become symptomatic or who do not show an appropriate increase in BP should probably be referred for surgery. [5] [22] The American Heart Association/American College of Cardiology recommends a transthoracic echocardiogram for asymptomatic patients with the following: [5] [22]
Grade 3 (loud) mid-systolic murmur Grade 2 (faint but easily detected) mid-systolic murmur and signs of cardiac dysfunction on examination.

Serial follow-up of asymptomatic patients found to have AS depends on the severity of stenosis and is as follows: [5] [22]
Mild stenosis: transthoracic echocardiogram every 3 to 5 years Moderate stenosis: transthoracic echocardiogram every 1 to 2 years Severe stenosis: transthoracic echocardiogram every year.

Following AVR, patients should have a complete physical examination 2 to 4 weeks after hospital discharge with attention to the presence or improvement of preoperative symptoms. A transthoracic echocardiogram should be performed as well to assess prosthetic valve function. In the absence of clinical deterioration, history and physical examination should be performed at least annually. Any change in clinical status should be evaluated with an echocardiogram, and patients with bioprosthetic valves should probably have an echocardiogram annually after 5 years even in the absence of symptoms to evaluate valve function. [5] [22] While the echocardiographic assessment of transcatheter aortic valve replacement (TAVR) prostheses is similar to the assessment of surgically-placed valves, current recommendations call for more frequent evaluations in patients with these new devices. [26] Patients with paravalvular aortic regurgitation have a higher risk of mortality and should be followed even more closely. [33]

Patient Instructions
Patients with prosthetic valves should be aware of the need for antibiotics prior to certain medical procedures (e.g., dental visits), and when uncertain, should ask medical professionals about the appropriateness of prophylaxis. [47] Patients must be aware that following AVR, they still have significant valve disease. In addition, other cardiovascular comorbidities frequently exist, and thus appropriate diet and exercise regimens should be adopted.

The return of preoperative symptoms needs to be brought to the attention of a medical professional as soon as they are noted so that appropriate evaluation can be performed. Online information from recommended websites may be helpful. [NHS Choices: Aortic valve replcement] (external link)

Complications
Complicationhide all

acute congestive heart failure see our comprehensive coverage of Acute exacerbation of congestive heart failure Heart failure occurs when the afterload burden created by the AS limits the ability of the left ventricle to pump blood to the body. Treatment of heart failure in AS is difficult. Diuretics and careful afterload reduction with vasodilators is the best option. Beta-blockers and calcium channel blockers should be used cautiously if at all because of their negative inotropic effects on the left ventricle. Therapy for symptomatic AS must address the underlying stenosis via surgical valve replacement or balloon valvuloplasty. sudden cardiac death in symptomatic patients see our comprehensive coverage of Cardiac arrest Sudden cardiac death due to ventricular arrhythmia is very rare in the asymptomatic patient (1%) but is a significant concern in symptomatic patients. [56] sudden cardiac death in asymptomatic patients see our comprehensive coverage of Cardiac arrest Sudden cardiac death due to ventricular arrhythmia is very rare in the asymptomatic patient (1%) but is a significant concern in symptomatic patients. [56] infection of prosthetic valve see our comprehensive coverage of Infective endocarditis Any prosthetic valve is at risk for infection and any fever or signs of valve dysfunction should prompt blood cultures and evaluation of valve function. thrombosis secondary to a mechanical valve Mechanical valves are subject to thrombosis and embolisation and require lifelong anticoagulation. re-stenosis If patients have a return of preoperative symptoms >10 years after replacement, re-stenosis should be considered. valve dehiscence

Suspected when patients develop new signs of heart failure or have a diastolic murmur of aortic regurgitation.

Last

Prognosis
For those patients with mild or moderate stenosis, the aortic valve area decreases on average by 0.1 cm^2/year and the mean gradient increases by 7 mmHg annually. [55] It is recommended that patients with moderate AS have an echocardiogram every 1 to 2 years, and those with mild AS have one every 3 to 5 years. [5] [22] It is important to note that the rate of progression is extremely variable and so the recommended follow-up periods may vary between individuals. The onset of symptoms is a significant milestone and portends a poor prognosis, with an average survival of only 2 to 3 years without surgery. Between 8% and 34% of symptomatic patients die suddenly. [56] It is thus essential that symptomatic patients be referred for surgical aortic valve replacement. Surgical replacement of the aortic valve is extremely effective therapy. Advances in prosthetic-valve design, cardiopulmonary bypass, surgical technique, and anaesthesia have steadily improved outcomes for aortic valve surgery. An analysis of the 2006 American Society of Thoracic Surgery STS database shows that during the previous decade, the mortality risk of isolated, aortic valve replacement decreased from 3.4% to 2.6%. For those <70 years at surgery, the mortality risk is 1.3%. [27] Patients who survive surgery have near-normal life expectancy, with relative survival at 5, 10, and 15 years of 99%, 85%, and 82%, respectively. [57][58] Nearly all patients have improvement in ejection fraction and heart failure symptoms, with the most significant benefit seen in those with more advanced preoperative symptoms. [5] [59][48] For those who do not improve, factors to consider are valve dysfunction, less-than-expected improvement in preoperative left ventricle function, valve-prosthesis mismatch, and other comorbid conditions. Patients at high risk from surgery may be offered surgery or transcatheter aortic valve replacement (TAVR), while patients deemed inoperable (i.e., surgical non-candidates) should be referred for TAVR if they have the potential to benefit. [26] In the PARTNER study comparing surgery and TAVR in high-risk patients, mortality and symptom reduction were similar at 2 years for each modality. [33] Peri-procedural risks varied at 30 days; vascular complications and neurologic events occurred more frequently after TAVR, while major bleeding and new-onset atrial fibrillation more commonly followed surgery. [34] At 2 years, echocardiographic improvements in valve area and

mean gradients were similar in both groups, but paravalvular aortic regurgitation was encountered more frequently after TAVR. [33] In patients deemed inoperable (i.e., surgical non-candidates), the PARTNER trial compared standard therapy, including balloon aortic valvuloplasty, with TAVR and found a 20% absolute reduction in mortality at 1 year in favour of TAVR. [36] These differences in survival were sustained at 2 years. [37] Heart failure symptoms improved between 30 days and 6 months, and were stable at 2 years, such that 83% of surviving TAVR patients were in New York Heart Association Class I/II, compared with only 8% at baseline. [37] [36] Patients treated with TAVR also showed significant improvements on assessments of health-related quality of life when compared to those receiving standard therapy. [38] Most patients with bicuspid aortic valves will require valve replacement at some point in their lives. After valve replacement, patients with bicuspid valves have a significant mortality benefit and improvement in symptoms. However, they remain at risk for aortic dissection and require serial follow-up for this potential complication.
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Bicuspid and trileaflet aortic valves with severe calcification following surgical excision From the collection of David Liff, MD, Emory University Hospital

The systolic crescendo-decrescendo murmur of aortic stenosis From the collection of David Liff, MD, Emory University Hospital

Transoesophageal echocardiogram showing the left ventricle outflow tract (LVOT), the aorta (Ao), and nearly immobile leaflets (arrows) of a severely stenotic aortic valve From the collection of David Liff, MD, Emory University Hospital

Balloon valvuloplasty fluoroscopy film that demonstrates valvuloplasty balloon inflated across a calcified aortic valve From the collection of David Liff, MD, Emory University Hospital

ECG showing changes associated with LVH From the collection of Melanie Everitt MD, Heart Failure & Transplantation Program, Primary Children's Medical Center, Salt Lake City, UT