Aortic Stenosis

Back

Practice Essentials

Aortic stenosis is the obstruction of blood flow across the aortic valve. Among symptomatic patients with medically treated moderate-to-severe aortic stenosis, mortality from the onset of symptoms is approximately 25% at 1 year and 50% at 2 years. Symptoms of aortic stenosis usually develop gradually after an asymptomatic latent period of 10-20 years.

Essential update: Potential causal association between LDL-C-related genetic variants and aortic valve disease

In a community-based study consisting of 6942 subjects with data on aortic valve calcium and more than 28,000 subjects with aortic stenosis (follow-up, >16 y), Smith et al found that genetic predisposition toward elevations in low-density lipoprotein cholesterol (LDL-C) (as indicated by genetic risk scores [GRSs])—but not elevated high-density lipoprotein cholesterol (HDL-C) or triglycerides GRSs—were associated with the presence of aortic valve calcium and the incidence of aortic stenosis.[1] The findings appear to suggest that early intervention aimed at reducing LDL-C levels may help to prevent aortic valve disease.[1]

Signs and symptoms

The classic triad of symptoms in patients with aortic stenosis is as follows[2] :

Systolic hypertension can coexist with aortic stenosis. However, a systolic blood pressure higher than 200 mm Hg is rare in patients with critical aortic stenosis.

In severe aortic stenosis, the carotid arterial pulse typically has a delayed and plateaued peak, decreased amplitude, and gradual downslope (pulsus parvus et tardus).

Other symptoms of aortic stenosis include the following:

See Clinical Presentation for more detail.

Diagnosis

The following studies are used in the diagnosis and assessment of aortic stenosis:

See Workup for more detail.

Management

The only definitive treatment for aortic stenosis is aortic valve replacement. The development of symptoms due to this condition provides a clear indication for replacement.[4, 5]

Emergency care

A patient presenting with uncontrolled heart failure should be treated supportively with oxygen, cardiac and oximetry monitoring, intravenous access, loop diuretics, nitrates (remembering the potential nitrate sensitivity of patients with aortic stenosis), morphine (as needed and tolerated), and noninvasive or invasive ventilatory support (as indicated). Patients with severe heart failure due to aortic stenosis that is resistant to medical management should be considered for urgent surgery.

Pharmacologic therapy

Agents used in the treatment of patients with aortic stenosis include the following:

Aortic valve replacement

According to American College of Cardiology (ACC)/American Heart Association (AHA) guidelines, candidates for aortic valve replacement include the following patients[6] :

Percutaneous balloon valvuloplasty

Percutaneous balloon valvuloplasty is used as a palliative measure in critically ill adult patients who are not surgical candidates or as a bridge to aortic valve replacement in critically ill patients.

See Treatment and Medication for more detail.

Image library


View Image

Calcific aortic stenosis (parasternal long-axis and short-axis views).

Background

Aortic stenosis is the obstruction of blood flow across the aortic valve. Aortic stenosis has several etiologies, including congenital (unicuspid or bicuspid valve), calcific (due to degenerative changes), and rheumatic. Degenerative calcific aortic stenosis is now the leading indication for aortic valve replacement. The favorable long-term outcome following aortic valve surgery and the relatively low operative risk emphasize the importance of an accurate and timely diagnosis (see Prognosis).

Stenotic valves are shown in the images below. Symptoms of aortic stenosis usually develop gradually after an asymptomatic latent period of 10-20 years. Exertional dyspnea or fatigue is the most common initial complaint. Ultimately, most patients experience the classic triad of chest pain, heart failure, and syncope (see History).

Two-dimensional (2D) Doppler echocardiography is the imaging modality of choice to diagnose and estimate the severity of aortic stenosis and localize the level of obstruction (see Workup). The only definitive treatment for aortic stenosis is aortic valve replacement (see Treatment and Management).

Go to Pediatric Valvar Aortic Stenosis, Pediatric Subvalvar Aortic Stenosis, and Pediatric Supravalvar Aortic Stenosis for more complete information on these topics.

Pathophysiology

When the aortic valve becomes stenotic, resistance to systolic ejection occurs and a systolic pressure gradient develops between the left ventricle and the aorta. This outflow obstruction leads to an increase in left ventricular (LV) systolic pressure. As a compensatory mechanism to normalize LV wall stress, LV wall thickness increases by parallel replication of sarcomeres, producing concentric hypertrophy. At this stage, the chamber is not dilated and ventricular function is preserved, although diastolic compliance is reduced.

Eventually, however, LV end-diastolic pressure (LVEDP) rises, which causes a corresponding increase in pulmonary capillary arterial pressures and a decrease in cardiac output due to diastolic dysfunction. The contractility of the myocardium may also diminish, which leads to a decrease in cardiac output due to systolic dysfunction. Ultimately, heart failure develops.

In most patients with aortic stenosis, LV systolic function is preserved and cardiac output is maintained for many years despite an elevated LV systolic pressure. Although cardiac output is normal at rest, it often fails to increase appropriately during exercise, which may result in exercise-induced symptoms.

Diastolic dysfunction may occur as a consequence of impaired LV relaxation and/or decreased LV compliance, as a result of increased afterload, LV hypertrophy, or myocardial ischemia. LV hypertrophy often regresses following relief of valvular (also called valvular) obstruction. However, some individuals develop extensive myocardial fibrosis, which may not resolve despite regression of hypertrophy.

In patients with severe aortic stenosis, atrial contraction plays a particularly important role in diastolic filling of the left ventricle. Thus, development of atrial fibrillation in aortic stenosis often leads to heart failure due to an inability to maintain cardiac output.

Increased LV mass, increased LV systolic pressure, and prolongation of the systolic ejection phase all elevate the myocardial oxygen requirement, especially in the subendocardial region. Although coronary blood flow may be normal when corrected for LV mass, coronary flow reserve is often reduced.

Myocardial perfusion is thus compromised by the relative decline in myocardial capillary density and by a reduced diastolic transmyocardial (coronary) perfusion gradient due to elevated LV diastolic pressure. Therefore, the subendocardium is susceptible to underperfusion, which results in myocardial ischemia.

Angina results from a concomitant increased oxygen requirement by the hypertrophic myocardium and diminished oxygen delivery secondary to diminished coronary flow reserve, decreased diastolic perfusion pressure, and relative subendocardial myocardial ischemia.

Etiology

Most cases of aortic stenosis are due to the obstruction at the valvular level. Common causes are summarized in Table 1.

Table 1. Common Causes of Aortic Stenosis Among Patients Requiring Surgery


View Table

See Table

Valvular aortic stenosis can be either congenital or acquired.

Congenital valvular aortic stenosis

Congenitally unicuspid, bicuspid, tricuspid, or even quadricuspid valves may be the cause of aortic stenosis. In neonates and infants younger than 1 year, a unicuspid valve can produce severe obstruction and is the most common anomaly in infants with fatal valvular aortic stenosis. In patients younger than 15 years, unicuspid valves are most frequent in cases of symptomatic aortic stenosis.

In adults who develop symptoms from congenital aortic stenosis, the problem is usually a bicuspid valve. Bicuspid valves do not cause significant narrowing of the aortic orifice during childhood. The altered architecture of the bicuspid aortic valve induces turbulent flow with continuous trauma to the leaflets, ultimately resulting in fibrosis, increased rigidity and calcification of the leaflets, and narrowing of the aortic orifice in adulthood.

A cohort study by Tzemos et al of 642 ambulatory adults with bicuspid aortic valves found that during the mean follow-up duration of 9 years, survival rates were not lower than for the general population. However, young adults with bicuspid aortic valve had a high likelihood of eventually requiring aortic valve intervention.[7]

Congenitally malformed tricuspid aortic valves with unequally sized cusps and commissural fusion (“functionally bicuspid” valves) can also cause turbulent flow leading to fibrosis and, ultimately, to calcification and stenosis. Clinical manifestations of congenital aortic stenosis in adults usually appear after the fourth decade of life.

Acquired valvular aortic stenosis

The main causes of acquired aortic stenosis include degenerative calcification and, less commonly, rheumatic heart disease.

Degenerative calcific aortic stenosis (also called senile calcific aortic stenosis) involves progressive calcification of the leaflet bodies, resulting in limitation of the normal cusp opening during systole. This represents a consequence of long-standing hemodynamic stress on the valve and is currently the most frequent cause of aortic stenosis requiring aortic valve replacement. The calcification may also involve the mitral annulus or extend into the conduction system, resulting in atrioventricular or intraventricular conduction defects.

Risk factors for degenerative calcific aortic stenosis include hypertension, hypercholesterolemia, diabetes mellitus, and smoking. The available data suggest that the development and progression of the disease are due to an active disease process at the cellular and molecular level that shows many similarities with atherosclerosis, ranging from endothelial dysfunction to, ultimately, calcification.[8]

In rheumatic aortic stenosis, the underlying process includes progressive fibrosis of the valve leaflets with varying degrees of commissural fusion, often with retraction of the leaflet edges and, in certain cases, calcification. As a consequence, the rheumatic valve often is regurgitant and stenotic. Coexistent mitral valve disease is common.

Other, infrequent causes of aortic stenosis include obstructive vegetations, homozygous type II hypercholesterolemia, Paget disease, Fabry disease, ochronosis, and irradiation.

It is worthwhile to note that although differentiation between tricuspid and bicuspid aortic stenosis is frequently made, it is often difficult to determine the number of aortic valve leaflets. A study comparing operatively excised aortic valve structure evaluation by cardiac surgeon versus pathologist found that valve structure determination was frequently incongruous.[9]

Epidemiology

Severe aortic stenosis is rare in infancy, occurring in 0.33% of live births, and is due to a unicuspid or bicuspid valve. Most patients with a congenitally bicuspid aortic valve who develop symptoms do not do so until middle age or later. Patients with rheumatic aortic stenosis typically present with symptoms after the sixth decade of life.

Aortic sclerosis (aortic valve calcification without obstruction to blood flow, considered a precursor of calcific degenerative calcific aortic stenosis) increases in incidence with age and is present in 29% of individuals older than 65 years and in 37% of individuals older than 75 years. In elderly persons, the prevalence of aortic stenosis is between 2% and 9%.

Degenerative calcific aortic stenosis usually manifests in individuals older than 75 years and occurs most frequently in males.[4]

Prognosis

Patients with severe aortic stenosis may be asymptomatic for many years despite the presence of severe LV outflow tract obstruction (LVOTO). LVOTOs have been associated with “high heritability.” One study suggests that 20% of patients with isolated LVOTO had an affected first-degree relative with undetected bicuspid aortic valves.[10]

Asymptomatic patients, even with critical aortic stenosis, have an excellent prognosis for survival, with an expected death rate of less than 1% per year; only 4% of sudden cardiac deaths in severe aortic stenosis occur in asymptomatic patients. A new proposed aortic stenosis grading classification that integrates valve area and flow-gradient patterns has been found to allow for better characterization of the clinical outcome among patients with asymptomatic severe aortic stenosis.[11]

Although the presence of low-gradient "severe stenosis" (defined as aortic valve area < 1.0 cm2 and mean gradient 40 mm Hg) is considered by some to be associated with a poor prognosis, the prospective Simvastatin and Ezetimibe in Aortic Stenosis (SEAS) study found that such patients have an outcome similar to that of patients with moderate stenosis.[12]

Among symptomatic patients with medically treated, moderate-to-severe aortic stenosis, mortality rates from the onset of symptoms are approximately 25% at 1 year and 50% at 2 years. More than 50% of deaths are sudden. In patients in whom the aortic valve obstruction remains unrelieved, the onset of symptoms predicts a poor outcome with medical therapy; the approximate time interval from the onset of symptoms to death is 1.5-2 years for heart failure, 3 years for syncope, and 5 years for angina.

Although the obstruction tends to progress more rapidly in degenerative calcific aortic valve disease than in congenital or rheumatic disease, predicting the rate of progression in individual patients is not possible. Catheterization and echocardiographic studies suggest that, on average, the valve area declines 0.1-0.3 cm2 per year; the systolic pressure gradient across the valve can increase by as much as 10-15 mm Hg per year. Obstruction progresses more rapidly in elderly patients with coronary artery disease and chronic renal insufficiency.

Patient Education

For patient education information, see eMedicineHealth's patient education article Angina Pectoris.

History

Aortic stenosis usually has an asymptomatic latent period of 10-20 years. During this time, the LV outflow obstruction and the pressure load on the myocardium gradually increase. Symptoms develop gradually. Exertional dyspnea is the most common initial complaint, even in patients with normal LV systolic function, and it often relates to abnormal LV diastolic function. In addition, patients may develop exertional chest pain, effort dizziness or lightheadedness, easy fatigability, and progressive inability to exercise. Ultimately, patients experience the classic triad of chest pain, heart failure, and syncope.[2]

Chest pain

Angina pectoris in patients with aortic stenosis is typically precipitated by exertion and relieved by rest. Thus, it may resemble angina from coronary artery disease.

Heart failure

Heart failure symptoms (ie, paroxysmal nocturnal dyspnea, orthopnea, dyspnea on exertion, and shortness of breath) may be due to systolic dysfunction from afterload mismatch, ischemia, or a separate cardiomyopathic process. Alternatively, diastolic dysfunction from LV hypertrophy or ischemia may also result in heart failure symptoms.

Syncope

Syncope from aortic stenosis often occurs upon exertion when systemic vasodilatation in the presence of a fixed forward stroke volume causes the arterial systolic blood pressure to decline. It also may be caused by atrial or ventricular tachyarrhythmias.

Syncope at rest may be due to transient ventricular tachycardia, atrial fibrillation, or (if calcification of the valve extends into the conduction system) atrioventricular block. Another cause of syncope is abnormal vasodepressor reflexes due to increased LV intracavitary pressure (vasodepressor syncope).

Syncope may be accompanied by convulsions.[13]

Patients with aortic stenosis may have a higher incidence of nitroglycerin-induced syncope than does the general population. Always consider aortic stenosis as a possible etiology for a patient who demonstrates particular hemodynamic sensitivity to nitrates.

Other manifestations

Gastrointestinal bleeding due to angiodysplasia (ie, Heyde syndrome[14] ) or other vascular malformations is present at a higher than expected frequency in patients with calcific aortic stenosis. These malformations usually resolve following aortic valve surgery.

Patients may present with manifestations of infective endocarditis (ie, fever, fatigue, anorexia, back pain, and weight loss). The risk of infective endocarditis is higher in younger patients with mild valvular deformity than in older patients with degenerated calcified aortic valves, but it can occur in either population. It can occur in patients of any age with hospital-acquired Staphylococcus aureus bacteremia.

Calcific aortic stenosis rarely may cause emboli of calcium to various organs, including the heart, kidney, and brain.

Physical Examination

In severe aortic stenosis, the carotid arterial pulse typically has a delayed and plateaued peak, decreased amplitude, and gradual downslope (pulsus parvus et tardus). However, in elderly individuals with rigid carotid vessels, this sign may not be present. A lag time may be present between the apical impulse and the carotid impulse.

Systolic hypertension can coexist with aortic stenosis. However, a systolic blood pressure higher than 200 mm Hg is rare in patients with critical aortic stenosis.

Pulsus alternans can occur in the presence of LV systolic dysfunction. The jugular venous pulse may show prominent a waves reflecting reduced right ventricular compliance consequent to hypertrophy of the interventricular septum.

A hyperdynamic LV is unusual and suggests concomitant aortic regurgitation or mitral regurgitation.

S1 is usually normal or soft. The aortic component of the second heart sound, A2, is usually diminished or absent, because the aortic valve is calcified and immobile and/or the aortic ejection is prolonged and it is obscured by the prolonged systolic ejection murmur. The presence of a normal or accentuated A2 speaks against the presence of severe aortic stenosis.

Paradoxical splitting of the S2 also occurs, resulting from late closure of A2. P2 may also be accentuated in the presence of secondary pulmonary hypertension.

An ejection click is common in children and young adults with congenital aortic stenosis, but it is rare in elderly individuals with acquired calcific aortic stenosis, in whom the cusps become immobile and severely calcified. This sound occurs approximately 40-60 milliseconds after the onset of S1 and is frequently heard best along the mid to lower left sternal border; it is often well transmitted to the apex and may be confused with a split S1.

A prominent S4 can be present and is due to forceful atrial contraction into a hypertrophied left ventricle. The presence of an S4 in a young patient with aortic stenosis indicates significant aortic stenosis, but with aortic stenosis in an elderly person, this is not necessarily true.

Systolic murmur

The classic crescendo-decrescendo systolic murmur of aortic stenosis begins shortly after the first heart sound. The intensity increases toward midsystole, then decreases, and the murmur ends just before the second heart sound. It is generally a rough, low-pitched sound that is best heard at the second intercostal space in the right upper sternal border. It is harsh at the base and radiates to 1 or both carotid arteries.

In elderly persons with calcific aortic stenosis, however, the murmur may be more prominent at the apex, because of radiation of its high-frequency components (Gallavardin phenomenon). This may lead to its misinterpretation as a murmur of mitral regurgitation. Accentuation of the aortic stenosis murmur following a long R-R interval (as in atrial fibrillation or following a premature beat) distinguishes it from the mitral regurgitation murmur, which usually does not change.

The intensity of the systolic murmur does not correspond to the severity of aortic stenosis; rather, the timing of the peak and the duration of the murmur corresponds to the severity of aortic stenosis. The more severe the stenosis, the longer the duration of the murmur and the more likely it peaks at late systole.

The murmur of valvular aortic stenosis is augmented upon squatting or following a premature beat; the murmur intensity is reduced during Valsalva strain. This is contrary to what occurs with hypertrophic obstructive cardiomyopathy, in which a Valsalva maneuver increases the intensity of the murmur.

When the left ventricle fails and cardiac output falls, the aortic stenosis murmur becomes softer and may be barely audible. Atrial fibrillation with short R-R intervals can also decrease the murmur intensity or make it inaudible.

Other findings

A high-pitched, diastolic blowing murmur may be present if the patient has associated aortic regurgitation.

Rarely, right ventricular failure with systemic venous congestion, hepatomegaly, and edema precede LV failure. This is probably due to the bulging of the interventricular septum into the right ventricle, with impedance in filling, elevated jugular venous pressure, and a prominent a wave (Bernheim effect).

Approach Considerations

Diagnostic studies in the emergency department should include electrocardiography (ECG), chest radiography, serum electrolyte levels, cardiac biomarkers, and a complete blood count (CBC). Arterial blood gas measurements are generally not necessary but may be obtained if hypoxemia or a mixed respiratory disease state is suspected.

Two-dimensional and Doppler echocardiography is the imaging modality of choice to diagnose and determine the severity of aortic stenosis.[5] In general, cardiac catheterization is not necessary to determine the severity of aortic stenosis. However, in instances in which clinical findings are not consistent with echocardiogram results, cardiac catheterization is recommended for further hemodynamic assessment.

Go to Imaging in Aortic Stenosis for more complete information on this topic.

Echocardiography

The American College of Cardiology/American Heart Association (ACC/AHA) recommendations from the ACC/AHA 2006 valvular heart disease guidelines for echocardiography in aortic stenosis are summarized below, in Table 2.[6] LV size, mass, and function should also be evaluated in each patient.

Table 2. ACC/AHA Recommendations for Echocardiography (Imaging, Spectral, and Color Doppler) in Aortic Stenosis


View Table

See Table

Two-dimensional transthoracic echocardiography can confirm the clinical diagnosis of aortic stenosis and provide specific data on LV function. The etiology of aortic stenosis (bicuspid, rheumatic, or degenerative calcific) may be assessed from the 2D echocardiographic, parasternal, short-axis view (see the image below). The structure and function of the other heart valves can also be assessed.


View Image

Calcific aortic stenosis (parasternal long-axis and short-axis views).

The following 3 echocardiographic findings are indicative of severe aortic stenosis:

Although the presence of aortic stenosis is readily diagnosed with 2D echocardiography, the severity of aortic stenosis cannot be judged based on the 2D echocardiographic images alone. Doppler echocardiography is an excellent tool for assessing the severity of aortic stenosis.

Using the modified Bernoulli equation, a maximum instantaneous and mean aortic valve gradient can be derived from the continuous-wave Doppler velocity across the aortic valve. In a laboratory with experienced personnel, Doppler-derived aortic valve gradients are accurate and reproducible and correlate well with those obtained during cardiac catheterization.

The transvalvular gradient is dependent on the severity of obstruction and the flow across the valve. In patients with low cardiac output, the valvular stenosis may be severe even though the transvalvular gradient is low. To overcome this problem, 2D Doppler echocardiography can also provide a reliable estimation of aortic valve area (AVA). The echocardiographic criteria for assessment of aortic stenosis severity are outlined below, in Table 3.

Table 3. Criteria for Determining Severity of Aortic Stenosis


View Table

See Table

The ACC/AHA 2006 Guidelines for the Management of Patients with Valvular Heart Disease include jet velocity (m/sec) in their criteria[6] :

According to the European Society of Cardiology (ESC)/European Association for Cardio-Thoracic Surgery (EACTS) 2012 guidelines on the management of valvular heart disease, the echocardiographic criteria for defining severe aortic stenosis also include valve area less than 1.0 cm2, mean gradient greater than 40 mm Hg, and maximum jet velocity greater than 4 m per second.[5]

Color Doppler valve analysis during transesophageal echocardiography (TEE) can be used to accurately diagnose bicuspid aortic valve in patients with severe symptomatic aortic stenosis, according to a prospective study of 51 patients. In detecting bicuspid aortic valve, color Doppler TEE had a sensitivity of 95.5%, a specificity of 96.5%, and a positive predictive value of 95.5%.[15]

The major limitation of Doppler echocardiography in assessing the severity of aortic stenosis is underestimation of the gradient if the beam is not parallel to the aortic stenosis velocity jet. Thus, in a patient with clinical features of severe aortic stenosis but echo/Doppler findings of mild to moderate aortic stenosis, further evaluation with repeat Doppler or cardiac catheterization may be required.

Rarely, Doppler may overestimate the severity of aortic stenosis in patients with severe anemia (hemoglobin < 8 g/dL), a small aortic root, or sequential stenoses in parallel (coexistent LV outflow tract [LVOT] and valvular obstruction).

Furthermore, echocardiographic calculation of AVA is highly dependent on accurate measurement of the diameter of the LVOT. In patients with poor transthoracic echocardiographic images, TEE may be used to measure the mean and peak gradient and a planimeter may be used to assess the AVA.

In patients who are potential candidates for transcatheter aortic valve replacement (see below), the role of echocardiography is critical. For this reason, the European Association of Echocardiography (EAE) and American Society of Echocardiography (ASE) have published recommendations for the use of echocardiography in patients undergoing transcatheter aortic valve replacement.[16]

Cardiac Catheterization and Coronary Arteriography

Cardiac catheterization provides an accurate measure of aortic stenosis and is an important tool, particularly in patients who have discrepant clinical and echocardiographic findings.[4] In general, if clinical findings are not consistent with Doppler echocardiogram results, cardiac catheterization is recommended for further hemodynamic assessment. The recommendations of the ACC/AHA 2006 valvular heart disease guidelines for cardiac catheterization in aortic stenosis are summarized below, in Table 4.[6]

Table 4. Recommendations for Cardiac Catheterization in Aortic Stenosis


View Table

See Table

The ESC/EACTS guidelines recommend restricting cardiac catheterization to use in patients in whom non-invasive evaluation is inconclusive or discordant with clinical findings.[5]

Measuring the LV end-diastolic and systolic volume and calculating the EF can quantitate the status of LV systolic pump function. However, EF may underestimate LV performance in the presence of the increased afterload associated with severe aortic stenosis. Since bolus administration of contrast may provoke hemodynamic compromise and assessment of LV function can usually be obtained via echocardiography, contrast ventriculography is rarely indicated.

Exclusion of coronary artery disease by coronary angiography is important in all patients older than 35 years who are being considered for valve surgery. Coronary angiography should also be performed in patients younger than 35 years if they have LV systolic dysfunction, symptoms or signs suggestive of coronary artery disease, or 2 or more risk factors for premature coronary artery disease, excluding sex. Generally, the incidence of associated coronary artery disease has been reported to be 50% in patients with aortic stenosis who are older than 50 years. Coronary angiography need not be performed in young patients with no atherosclerotic risk factors and in circumstances where the risk involved outweighs the benefits.[5]

Radionuclide Ventriculography

Radionuclide studies to evaluate myocardial perfusion at rest and during exertion and exercise may be considered as part of the complete workup of aortic stenosis. Radionuclide ventriculography may provide information on LV function, including LVEF, ESV, and EDV. Perform these tests cautiously on symptomatic patients.[17]

Exercise Stress Testing

Exercise stress testing is contraindicated in symptomatic patients with severe aortic stenosis, but it may be considered in asymptomatic patients with severe aortic stenosis. The ACC/AHA 2006 valvular heart disease guidelines state that exercise testing may be considered in asymptomatic patients (class IIb recommendation), and recommend that exercise testing not be performed in symptomatic patients with aortic stenosis without specifying severity (class III).[6] In asymptomatic patients, stress testing has been shown to be a low-risk procedure when it is performed under strict surveillance.[17]

Closely monitored exercise stress testing may be of value to assess exercise capacity in asymptomatic patients. Abnormal results may prove greater disability than the patient would admit. In addition to watching for symptoms on the treadmill, one should also look for hemodynamic abnormalities, such as blood pressure decreases or failure to increase blood pressure normally, which can occur in the absence of symptoms. In this setting, the test is not used to screen for coronary disease.

Provocative stress testing is used in cases when the severity of the aortic stenosis is uncertain because of a small stroke volume and a small mean aortic valve gradient (low-gradient aortic stenosis). Infusion of an inotropic agent such as dobutamine, which results in an increase in stroke volume and heart rate, is usually helpful in establishing the correct diagnosis. Cardiac output and LV and aortic pressures are measured simultaneously and AVA is calculated before and during dobutamine infusion.

In patients with an initially low-pressure gradient but severe aortic stenosis, the measured AVA does not change with an intravenous dobutamine infusion, but the mean-pressure gradient increases significantly. In contrast, in patients who have a low cardiac output due to concomitant myocardial dysfunction rather than due to severe aortic stenosis alone, a small increase in the measured AVA and the aortic valve gradient usually occurs with dobutamine infusion.

Investigational Imaging Modalities

Three-dimensional (3D) volume quantification of aortic valve calcification using multislice computed tomography (CT) scanning demonstrates a close, nonlinear relationship to echocardiographic parameters for the severity of aortic stenosis.[5, 18] This method is not yet clinically validated.

In a study by Shah et al that compared multidetector CT scanning with TEE, multidetector CT scanning was found to be an accurate modality for determining aortic valve measurements in patients with aortic stenosis.[19]

Cardiac magnetic resonance imaging (MRI) has also been investigated for assessment of aortic stenosis. AVA measurements made with cardiac MRI have shown excellent correlation with those made with Doppler echocardiography. This method is not yet clinically validated.

Chest Radiography

Even in the presence of significant aortic stenosis, the cardiac size often is normal, with rounding of the LV border and apex. Poststenotic dilatation of the ascending aorta is common.

On lateral views, aortic valve calcification is found in almost all adults with hemodynamically significant aortic stenosis. Although its absence on fluoroscopy in individuals older than 35 years rules out severe valvular aortic stenosis, its presence does not prove severe obstruction in individuals older than 60 years.

The left atrium may be slightly enhanced, and pulmonary venous hypertension may be seen. In later, more severe stages of aortic stenosis, radiographic signs of left atrial enlargement, pulmonary artery enlargement, right-sided enlargement, calcification of the aortic valve, and pulmonary congestion may be evident.

Electrocardiography

Generally, ECG is not a reliable test for aortic stenosis. The results vary widely in patients with this disorder and overlap with other cardiac conditions.

Although the ECG findings may be entirely normal, the principal finding is left ventricular hypertrophy (LVH), which is found in 85% of patients with severe aortic stenosis; however, its absence does not preclude critical aortic stenosis. Patients with significant aortic stenosis who may not show clear ECG evidence of ventricular hypertrophy include elderly persons with significant myocardial fibrosis and adolescents, who may experience ST-segment changes before QRS changes.

T-wave inversion and ST-segment depression in leads with predominantly positive QRS complexes are common. ST depression exceeding 0.3 mV in patients with aortic stenosis indicates LV strain and suggests severe LVH. Occasionally, a septal pseudoinfarct pattern can be seen. Left atrial enlargement with a preterminal negative p wave in lead V1 is noted in 80% of cases of severe isolated aortic stenosis. The presence of left atrial enlargement suggests an associated mitral valve process.

The correlation between absolute voltages in precordial leads and the severity of obstruction, unlike in children with congenital aortic stenosis, is poor in adults.

The rhythm usually is normal sinus. Atrial fibrillation can be seen at late stages or as a consequence of coexistent MV disease or hyperthyroidism.

Extension of calcification into the conduction system can cause atrioventricular or intraventricular block in 5% of cases of aortic stenosis. Approximately 10% of all cases of left anterior fascicular block are secondary to calcific aortic valve disease. Ambulatory ECG monitoring frequently shows complex ventricular arrhythmias, particularly in cases with myocardial dysfunction.

While the degree of severity of changes on a single ECG does not correlate well with the degree of hemodynamic compromise, serial ECGs performed over time (months to years) can be valuable in demonstrating the progression of the disease.

B-type Natriuretic Peptide

B-type natriuretic peptide (BNP) may provide incremental prognostic information in predicting symptom onset in asymptomatic patients with severe aortic stenosis.[3] A high or steadily rising BNP may predict the short-term need for valve replacement in asymptomatic, severe aortic stenosis. Preoperative BNP provides prognostic information on postoperative outcome.[20] Go to Natriuretic Peptides in Congestive Heart Failure for more complete information on this topic.

Approach Considerations

The only definitive treatment for aortic stenosis is aortic valve replacement. The development of symptoms due to aortic stenosis provides a clear indication for replacement. For patients who are not candidates for aortic replacement, percutaneous aortic balloon valvuloplasty may provide some symptom relief.[4, 5]

Medical treatment (such as diuretic therapy) in aortic stenosis may provide temporary symptom relief but is generally not effective long term.

In truly asymptomatic patients with severe aortic stenosis, the issue of valve replacement is less clear.[5]

Emergency Department Care

Prehospital and emergency department management is focused on acute exacerbations of the symptoms of aortic stenosis. As always, assess and address airway, breathing, and circulation. If the patient is in cardiopulmonary arrest, perform resuscitation according to the recommendations of the AHA in their Advanced Cardiac Life Support guidelines. In patients with acute symptoms, hospital admission, telemetry/intensive care unit admission, and cardiology consultation all should be considered.

A patient presenting with uncontrolled heart failure should be treated supportively with oxygen, cardiac and oximetry monitoring, intravenous access, loop diuretics, nitrates (remembering the potential nitrate sensitivity of patients with aortic stenosis), morphine (as needed and tolerated), and noninvasive or invasive ventilatory support (as indicated). Patients with severe heart failure due to aortic stenosis that is resistant to medical management should be considered for urgent surgery.

A patient presenting with angina pectoris requires monitoring and studies as listed above. Measures should be taken to relieve the chest discomfort. This may include the administration of nitrates, oxygen, and morphine. However, nitroglycerin-induced syncope occurs more often in patients with aortic stenosis than in those without aortic stenosis. This information should be obtained through the history at presentation.

Syncope in the face of aortic stenosis should be assessed and treated as in any patient presenting with a syncopal episode.

Atrial fibrillation in the setting of aortic stenosis is considered a medical emergency, and sinus rhythm should be restored urgently in patients who are hemodynamically unstable. Associated symptoms also should be treated urgently.

Percutaneous Balloon Valvuloplasty

Percutaneous balloon valvuloplasty is used as a palliative measure in critically ill adult patients who are not surgical candidates or as a bridge to aortic valve replacement in critically ill patients. The high rate of restenosis and the absence of a mortality benefit preclude its use as a definitive treatment method in adults with severe aortic stenosis.

Valvuloplasty can be considered in cases of severe heart failure or cardiogenic shock for the following patients:

In critically ill patients, the mortality rate associated with the procedure is 3-7%. Another 6% develop serious complications, including perforation, myocardial infarction, and severe aortic regurgitation.

In children, adolescents, and young adults with congenital aortic stenosis, percutaneous balloon valvuloplasty carries a mortality risk of 1% and may be an alternative to surgical valvotomy. The risk of causing significant aortic regurgitation is 10%. Although exercise restriction is sometimes recommended to avoid the risk of sudden unexpected death for some patients with congenital aortic stenosis, a recent study by Brown et al suggests that sudden unexpected death is extremely rare following balloon valvuloplasty, and the study found no beneficial effect for exercise restriction after the procedure is performed.[21]

The best results from valvuloplasty are obtained in the patients with a commissural bicuspid aortic valve, in whom a 60-70% reduction in gradient and a 60% increase in the AVA can be expected.

Restenosis is common, particularly in patients with unicuspid valves or with valves affected by severe dysplasia (>60% at 6 mo, virtually 100% at 2 y). However, repeat procedures have been shown to provide a median survival rate of approximately 3 years and to maintain clinical improvement.[22]

Aortic Valve Replacement

The recommendations of the ACC/AHA 2006 valvular heart disease guidelines for aortic valve replacement in patients with valvular aortic stenosis are summarized below, in Table 5.[6] In most adults with symptomatic, severe aortic stenosis, aortic valve replacement is the surgical treatment of choice. If concomitant coronary disease is present, aortic valve replacement and coronary artery bypass graft (CABG) should be performed simultaneously.

Table 5. Recommendations for Aortic Valve Replacement in Aortic Stenosis


View Table

See Table

Successful aortic valve replacement produces substantial clinical and hemodynamic improvement in patients with aortic stenosis, including octogenarians. According to both the ACC/AHA and ESC/EACTS guidelines[5, 6] , aortic valve replacement should be performed in all symptomatic patients with severe aortic stenosis, regardless of LV function, as survival is better with surgical treatment than with medical treatment.

Aortic valve replacement is also recommended in asymptomatic patients with severe aortic stenosis and LV dysfunction. Improvement in EF invariably occurs over the following 6 months, and increased LV mass tends to decrease within 18 months postoperatively.

In 2014, the ACC/AHA released updated guidelines on the management of valvular heart disease Recommendations include the following[23, 24] :

Guidelines for aortic stenosis include the following[23, 24] :

Bioprosthetic and mechanical valves

The choice of prosthesis is determined by the anticipated longevity of the patient and his/her ability to tolerate anticoagulation.[25]

Stassano et al found that bioprosthetic aortic valves were significantly less durable than were mechanical valves. In a prospective, randomized study of 310 patients aged 55-70 years, followup at 13 years showed that valve failures and reoperations were more frequent in the bioprosthesis group than in the mechanical prosthesis group. However, there were no differences between the 2 types of valves regarding the rates of survival, thromboembolism, bleeding, endocarditis, and major adverse prosthesis-related events.[26]

The surgical mortality risk in patients with normal LV systolic function and no other comorbid conditions is less than 5%. Risk factors for increased operative mortality include the following:

Overall, the 5-year survival rate in all adults after aortic valve replacement is 80-94%, and the 10-year survival rate is 68-89%. Risk factors for late death include the following:

Ross procedure

The Ross procedure is another option in young patients as an initial procedure or for reoperation after prior valvotomy. In this procedure, the patient's own pulmonary valve and main pulmonary artery are transplanted to the aortic position, with reimplantation of coronary arteries. A homograft is placed in the pulmonary position. Its durability may be better than tissue valves. However, the Ross procedure is technically demanding and results at different centers have been mixed.

Percutaneous transcatheter valve replacement

Many patients with severe aortic stenosis and coexisting conditions are not candidates for surgical replacement of the aortic valve. Studies have suggested that percutaneous transcatheter aortic-valve replacement (TAVR) with a balloon-expandable bovine pericardial valve is a less invasive option for these high-risk patients.[5, 27, 28] In a study comparing TAVR (via a transfemoral or a transapical approach) and surgical replacement in patients who were candidates for valve replacement but considered to be high risk, survival at 1 year was similar for both procedures.[29] However, important differences in periprocedural risks were observed; major vascular complications and stroke were more frequent with TAVR, whereas major bleeding and new-onset atrial fibrillation were more frequent with surgical valve replacement.

A comprehensive literature review by Daneault evaluated the incidence of stroke after surgical and transcatheter treatment for aortic stenosis. The risk of stroke for the general population after aortic valve replacement was 1.5% (2-4% in higher risk and elderly patients). The rate after transcatheter treatment was 1.5-6%. This review shows a trend for more strokes in the transcatheter group.[30]

In the more recent Placement of Aortic Transcatheter Valves (PARTNER) trial, inoperable patients with severe aortic stenosis had improved survival with transcatheter aortic valve replacement (TAVR) compared with medical management.[31] In high-risk patients, survival was similar with TAVR and surgical aortic valve replacement. In all the patient cohorts, low flow (stroke volume index ≤35 mL/m2) was an independent predictor of mortality, whereas low ejection fraction and mean gradient were not.[31]

Also in the PARTNER trial, patients with critical aortic stenosis after either surgical aortic valve replacement (SAVR) or TAVR showed decreased aortic valve gradients and increased effective orifice area (EOA) on echocardiography through 2 years of follow-up.[32] Univariate postimplantation echocardiographic predictors of death in the TAVR group were as follows:

In the SAVR group, the predictors of death were as follows:

In another randomized study, TAVR using a self-expanding transcatheter aortic-valve bioprosthesis (CoreValve) was associated with a significantly higher survival rate at 1 year follow-up than surgical aortic-valve replacement.[33, 34] The study consisted of 795 patients with severe aortic stenosis who were at increased surgical risk. The rate of death from any cause at 1 year was 14.2% in the TAVR group and 19.1% in the surgical group (P = 0.04). The risk of stroke at 30 days was 4.9% with TAVR and 6.2% with surgery.[33, 34]

In June 2014, the FDA widened the indication for the self-expanding transcatheter aortic-valve bioprosthesis CoreValve to include patients with symptomatic severe aortic stenosis who are at high risk for surgery.[35, 36] The original indication approved in January 2014 was for patients considered at extreme risk and thus not surgical candidates.[35]

Approval for the expanded indication was based on data from the head-to-head High-Risk Study of the CoreValve US Pivotal Trial, in which patients who underwent transcatheter aortic valve replacement (TAVR) with CoreValve had a significantly higher 1-year survival rate (85.8%) compared with those who underwent surgical valve replacement (80.9%).[35, 36] The rates of stroke were low and similar between the groups; however, relative to those who received a surgical valve, rates of major adverse cardiovascular/cerebral events were significantly better at 1 year and overall hemodynamic performance was better at all time points in those who underwent TAVR with CoreValve.[36]

Medical Treatment

The medical treatment options are limited in symptomatic patients with aortic stenosis who are not candidates for surgery. In patients with pulmonary congestion, cautious use of digitalis, diuretics, and angiotensin-converting enzyme (ACE) inhibitors might attempted, whereas beta-blockers might be used if the predominant symptom is angina. In any case, excessive decrease in preload or systemic arterial blood pressure should be avoided.

Vasodilators may be used for heart failure and for hypertension but should also be employed with extreme caution to avoid critically reducing preload or systemic arterial blood pressure in a patient with significant aortic stenosis.

Severe hypertension is frequently seen in the elderly patient with aortic stenosis and should be treated, because it causes an additional increase in vascular afterload. Treatment should follow the guidelines set out in the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure.[37] Reducing the blood pressure to normal levels is advisable, but hypotension must be avoided.[5]

The ESC/EACTS guidelines recommend that patients with heart failure symptoms who are not suitable candidates for surgery or transcatheter aortic valve implantation may be treated with digoxin, diuretics, ACE inhibitors, or angiotensin receptor blockers.[5]

Endocarditis prophylaxis

Antibiotic prophylaxis for the prevention of bacterial endocarditis is no longer recommended in patients with valvular aortic stenosis.[38]

Activity

Patients with mild aortic stenosis can lead a normal life. In cases of moderate aortic stenosis, moderate to severe physical exertion and competitive sports should be avoided.

Prevention/Deterrence

Although small, observational studies have suggested that statin use can reduce aortic valve leaflet calcification and delay the progression of aortic stenosis severity,[39] 3 randomized, double-blind, placebo controlled trials of almost 2200 patients showed that intensive lipid-lowering therapy does not halt the progression of calcific aortic stenosis or induce its regression.[40, 41, 42]

Complications

Possible complications of aortic stenosis include the following:

Consultations

Consultation with a cardiologist or cardiothoracic surgeon is appropriate.

Long-Term Monitoring

The frequency of the follow-up visits in asymptomatic patients is determined by the severity of aortic stenosis and by the presence of comorbid conditions.

In patients with mild aortic stenosis, yearly history and physical examination and an echocardiogram every 3-5 years are appropriate.

Patients with moderate or severe aortic stenosis should be examined twice yearly and whenever they develop symptoms that are potentially attributable to aortic stenosis.

In patients with moderate aortic stenosis, echocardiograms should be performed every 2 years, whereas in asymptomatic patients with severe aortic stenosis, yearly echocardiograms are recommended.

Following aortic valve replacement, every patient should undergo echocardiographic examination after recovery. Thereafter, an examination is recommended whenever new symptoms develop that are attributable to a potential valvular dysfunction.

Patients with mechanical valves should receive lifelong anticoagulation with warfarin and should undergo periodic screening of their anticoagulation status.

The 2011 ACCF/AHA/HRS Focused Update on the Management of Patients With Atrial Fibrillation (Update on Dabigatran) from the ACC Foundation (ACCF)/AHA/Heart Rhythm Society (HRS) states that the new anticoagulant dabigatran is useful as an alternative to warfarin in patients with atrial fibrillation. Dabigatran has not been studied in patients with atrial fibrillation and valvular heart disease and is not approved by the FDA for this population.[43]

Medication Summary

Treatment of valvular aortic stenosis is interventional. Medical treatment in aortic stenosis essentially is reserved for patients who have complications of the disorder, such as heart failure, infective endocarditis, hypertension, or arrhythmias.

The medical treatment options are limited in symptomatic patients with aortic stenosis who are not candidates for surgery. In patients with pulmonary congestion, cautious use of digitalis, diuretics, and angiotensin-converting enzyme (ACE) inhibitors might be attempted, whereas beta-blockers might be used if the predominant symptom is angina.

Antibiotic prophylaxis for the prevention of bacterial endocarditis is no longer recommended in patients with valvular aortic stenosis.[38]

Esmolol (Brevibloc)

Clinical Context:  Esmolol is an ultra–short-acting that selectively blocks beta1-receptors with little or no effect on beta2-receptor types. It is particularly useful in patients with elevated arterial pressure, especially if surgery is planned.

Metoprolol (Lopressor, Toprol XL)

Clinical Context:  Metoprolol is a selective beta1-adrenergic receptor blocker that decreases the automaticity of contractions. During intravenous (IV) administration, carefully monitor blood pressure (BP), heart rate, and electrocardiogram (ECG).

Class Summary

The medical treatment options are limited in symptomatic patients with aortic stenosis who are not candidates for surgery. Beta-blockers may be used if the predominant symptom is angina.

Digoxin (Lanoxin)

Clinical Context:  Digoxin enhances myocardial contractility by inhibition of Na+/K+ ATPase, a cell membrane enzyme that extrudes sodium and brings potassium into the myocyte. The resulting increase in intracellular sodium stimulates the sodium-calcium exchanger in the cell membrane, which extrudes sodium and brings in calcium, leading to an increase in intracellular calcium in the sarcoplasmic reticulum of cardiac cells, thereby increasing the contractility of myocytes.

Class Summary

Cardiac glycosides slow AV nodal conduction primarily by increasing vagal tone. Patients with aortic stenosis who are not candidates for surgery and present with pulmonary congestion may be treated with digoxin. Digoxin can also be used as an inotropic agent to control the ventricular rate in patients with atrial fibrillation.

Furosemide (Lasix)

Clinical Context:  Furosemide increases the excretion of water by interfering with the chloride-binding co-transport system, which, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and the distal renal tubule.

Bumetanide (Bumex)

Clinical Context:  Bumetanide increases the excretion of water by interfering with chloride-binding co-transport system, which, in turn, inhibits sodium, potassium, and chloride reabsorption in the ascending loop of Henle. These effects increase urinary excretion of sodium, chloride, and water, resulting in profound diuresis. Renal vasodilation occurs following administration, renal vascular resistance decreases, and renal blood flow is enhanced.

Class Summary

Loop diuretics act on the ascending limb of the loop of Henle, inhibiting the reabsorption of sodium and chloride. Prehospital and emergency department management is focused on acute exacerbations of the symptoms of aortic stenosis. A patient presenting with uncontrolled heart failure should be treated supportively with loop diuretics.

Captopril (Capoten)

Clinical Context:  Captopril prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

Enalapril (Vasotec)

Clinical Context:  Enalapril prevents the conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion. It helps control blood pressure and proteinuria. Enalapril decreases pulmonary-to-systemic flow ratio in the catheterization laboratory and increases systemic blood flow in patients with relatively low pulmonary vascular resistance.

Class Summary

These agents are competitive inhibitors of angiotensin-converting enzyme (ACE). They reduce angiotensin II levels, thus decreasing aldosterone secretion.

Morphine sulfate (MS Contin, Astramorph, Avinza)

Clinical Context:  Morphine is a drug of choice for analgesia due to reliable and predictable effects and safety profile. A patient presenting with uncontrolled heart failure due to aortic stenosis should be treated supportively with morphine.

Class Summary

Opioid analgesics such as morphine act by binding to opioid receptors on neurons distributed throughout the nervous system and immune system. They can also help patient anxiety, distress, and dyspnea.

Author

Xiushui (Mike) Ren, MD, Cardiologist, The Permanente Medical Group; Associate Director of Research, Cardiovascular Diseases Fellowship, California Pacific Medical Center

Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD, Professor and Executive Vice Chairman, Department of Medicine, Director, Office of Educational Programs, University of Texas Health Science Center at San Antonio

Disclosure: Nothing to disclose.

Additional Contributors

Jerry Balentine, DO Professor of Emergency Medicine, New York College of Osteopathic Medicine; Executive Vice President, Chief Medical Officer, Attending Physician in Department of Emergency Medicine, St Barnabas Hospital

Jerry Balentine, DO is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American College of Physician Executives, American Osteopathic Association, and New York Academy of Medicine

Disclosure: Nothing to disclose.

Edward Bessman, MD, MBA Chairman and Clinical Director, Department of Emergency Medicine, John Hopkins Bayview Medical Center; Assistant Professor, Department of Emergency Medicine, Johns Hopkins University School of Medicine

Edward Bessman, MD, MBA is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

David FM Brown, MD Associate Professor, Division of Emergency Medicine, Harvard Medical School; Vice Chair, Department of Emergency Medicine, Massachusetts General Hospital

David FM Brown, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Steven J Compton, MD, FACC, FACP, FHRS Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals

Steven J Compton, MD, FACC, FACP, FHRS is a member of the following medical societies: Alaska State Medical Association, American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, and Heart Rhythm Society

Disclosure: Nothing to disclose.

Daniel P Lombardi, DO Clinical Assistant Professor, New York College of Osteopathic Medicine; Attending Physician, Associate Department Director and Program Director, Department of Emergency Medicine, St Barnabas Hospital

Daniel P Lombardi, DO is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, and American Osteopathic Association

Disclosure: Nothing to disclose.

John A McPherson, MD, FACC, FAHA, FSCAI Associate Professor of Medicine, Division of Cardiovascular Medicine, Director of Cardiovascular Intensive Care Unit, Vanderbilt Heart and Vascular Institute

John A McPherson, MD, FACC, FAHA, FSCAI is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, Society for Cardiac Angiography and Interventions, Society of Critical Care Medicine, and Tennessee Medical Association

Disclosure: Abbott Vascular Corp. Consulting fee Consulting

Bekir H Melek, MD, FACC Assistant Professor of Clinical Medicine, Department of Medicine, Section of Cardiology, Tulane University School of Medicine

Disclosure: Nothing to disclose.

Gary Setnik, MD Chair, Department of Emergency Medicine, Mount Auburn Hospital; Assistant Professor, Division of Emergency Medicine, Harvard Medical School

Gary Setnik, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: SironaHealth Salary Management position; South Middlesex EMS Consortium Salary Management position; ProceduresConsult.com Royalty Other

James V Talano, MD, MM, FACC Director of Cardiovascular Medicine, SWICFT Institute

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

  1. Smith JG, Luk K, Schulz CA, et al. Association of low-density lipoprotein cholesterol-related genetic variants with aortic valve calcium and incident aortic stenosis. JAMA. Nov 5 2014;312(17):1764-71. [View Abstract]
  2. Tintinalli JE, Kelen GD, Stapczynski JS, eds. Valvular emergencies. In: 6th ed. Emergency Medicine: A Comprehensive Study Guide. New York: McGraw-Hill; 2004:54.
  3. Bergler-Klein J. Natriuretic peptides in the management of aortic stenosis. Curr Cardiol Rep. Mar 2009;11(2):85-93. [View Abstract]
  4. Townsend CM, et al. Sabiston Textbook of Surgery. 18th ed. Saunders; 2008:1841-1844..
  5. Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Barón-Esquivias G, Baumgartner H, et al. Guidelines on the management of valvular heart disease (version 2012): The Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. Oct 2012;33(19):2451-96. [View Abstract]
  6. [Guideline] Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing Committee to Revise the 1998 guidelines for the management of patients with valvular heart disease) developed in collaboration with the Society of Cardiovascular Anesthesiologists endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. J Am Coll Cardiol. Aug 1 2006;48(3):e1-148. [View Abstract]
  7. Tzemos N, Therrien J, Yip J, Thanassoulis G, Tremblay S, Jamorski MT, et al. Outcomes in adults with bicuspid aortic valves. JAMA. Sep 17 2008;300(11):1317-25. [View Abstract]
  8. Hughes BR, Chahoud G, Mehta JL. Aortic stenosis: is it simply a degenerative process or an active atherosclerotic process?. Clin Cardiol. Mar 2005;28(3):111-4. [View Abstract]
  9. Roberts WC, Vowels TJ, Ko JM. Comparison of interpretations of valve structure between cardiac surgeon and cardiac pathologist among adults having isolated aortic valve replacement for aortic valve stenosis (+/- aortic regurgitation). Am J Cardiol. Apr 15 2009;103(8):1139-45. [View Abstract]
  10. Kerstjens-Frederikse WS, Du Marchie Sarvaas GJ, et al. Left ventricular outflow tract obstruction: should cardiac screening be offered to first-degree relatives?. Heart. Aug 2011;97(15):1228-32. [View Abstract]
  11. Lancellotti P, Magne J, Donal E, et al. Clinical outcome in asymptomatic severe aortic stenosis insights from the new proposed aortic stenosis grading classification. J Am Coll Cardiol. Jan 17 2012;59(3):235-43. [View Abstract]
  12. Jander N, Minners J, Holme I, et al. Outcome of patients with low-gradient "severe" aortic stenosis and preserved ejection fraction. Circulation. Mar 1 2011;123(8):887-95. [View Abstract]
  13. Rodrigues Tda R, Sternick EB, Moreira Mda C. Epilepsy or syncope? An analysis of 55 consecutive patients with loss of consciousness, convulsions, falls, and no EEG abnormalities. Pacing Clin Electrophysiol. Jul 2010;33(7):804-13. [View Abstract]
  14. Topol EJ, Califf RM, et al, eds. Aortic valve disease. In: Textbook of Cardiovascular Medicine. Section Two. 3rd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2007:Chap 23.
  15. Zegdi R, Ciobotaru V, Huerre C, Allam B, Bouabdallaoui N, Berrebi A, et al. Detecting aortic valve bicuspidy in patients with severe aortic valve stenosis: high diagnostic accuracy of colour Doppler transoesophageal echocardiography. Interact Cardiovasc Thorac Surg. Jan 2013;16(1):16-20. [View Abstract]
  16. Zamorano JL, Badano LP, Bruce C, et al. EAE/ASE recommendations for the use of echocardiography in new transcatheter interventions for valvular heart disease. Eur Heart J. Sep 2011;32(17):2189-214. [View Abstract]
  17. Piérard LA, Lancellotti P. Stress testing in valve disease. Heart. Jun 2007;93(6):766-72. [View Abstract]
  18. Messika-Zeitoun D, Aubry MC, Detaint D, Bielak LF, Peyser PA, Sheedy PF, et al. Evaluation and clinical implications of aortic valve calcification measured by electron-beam computed tomography. Circulation. Jul 20 2004;110(3):356-62. [View Abstract]
  19. Shah RG, Novaro GM, Blandon RJ, Whiteman MS, Asher CR, Kirsch J. Aortic valve area: meta-analysis of diagnostic performance of multi-detector computed tomography for aortic valve area measurements as compared to transthoracic echocardiography. Int J Cardiovasc Imaging. Aug 2009;25(6):601-9. [View Abstract]
  20. Bergler-Klein J, Klaar U, Heger M, Rosenhek R, Mundigler G, Gabriel H, et al. Natriuretic peptides predict symptom-free survival and postoperative outcome in severe aortic stenosis. Circulation. May 18 2004;109(19):2302-8. [View Abstract]
  21. Brown DW, Dipilato AE, Chong EC, Gauvreau K, McElhinney DB, Colan SD, et al. Sudden unexpected death after balloon valvuloplasty for congenital aortic stenosis. J Am Coll Cardiol. Nov 30 2010;56(23):1939-46. [View Abstract]
  22. Agarwal A, Kini AS, Attanti S, Lee PC, Ashtiani R, Steinheimer AM, et al. Results of repeat balloon valvuloplasty for treatment of aortic stenosis in patients aged 59 to 104 years. Am J Cardiol. Jan 1 2005;95(1):43-7. [View Abstract]
  23. Wood S. New valve guidelines offer staging, risk-scoring advice. Medscape Medical News [serial online]. March 5, 2014;Accessed March 8, 2014. Available at http://www.medscape.com/viewarticle/821526
  24. Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, et al. 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. Mar 3 2014;[View Abstract]
  25. Rahimtoola SH. Choice of prosthetic heart valve in adults an update. J Am Coll Cardiol. Jun 1 2010;55(22):2413-26. [View Abstract]
  26. [Best Evidence] Stassano P, Di Tommaso L, Monaco M, Iorio F, Pepino P, Spampinato N, et al. Aortic valve replacement: a prospective randomized evaluation of mechanical versus biological valves in patients ages 55 to 70 years. J Am Coll Cardiol. Nov 10 2009;54(20):1862-8. [View Abstract]
  27. Leon MB, Smith CR, Mack M, Miller DC, Moses JW, Svensson LG, et al. Transcatheter aortic-valve implantation for aortic stenosis in patients who cannot undergo surgery. N Engl J Med. Oct 21 2010;363(17):1597-607. [View Abstract]
  28. Clavel, MA, et al. Comparison Between Transcatheter and Surgical Prosthetic Valve Implantation in Patients With Severe Aortic Stenosis and Reduced Left Ventricular Ejection Fraction. Circulation. Nov 9 2010Vol;. 122 No.19.
  29. Smith CR, Leon MB, Mack MJ, et al. Transcatheter versus surgical aortic-valve replacement in high-risk patients. N Engl J Med. Jun 9 2011;364(23):2187-98. [View Abstract]
  30. Daneault B, Kirtane AJ, Kodali SK, et al. Stroke associated with surgical and transcatheter treatment of aortic stenosis: a comprehensive review. J Am Coll Cardiol. Nov 15 2011;58(21):2143-50. [View Abstract]
  31. Herrmann HC, Pibarot P, Hueter I, Gertz ZM, Stewart WJ, Kapadia S, et al. Predictors of Mortality and Outcomes of Therapy in Low-Flow Severe Aortic Stenosis: A Placement of Aortic Transcatheter Valves (PARTNER) Trial Analysis. Circulation. Jun 11 2013;127(23):2316-26. [View Abstract]
  32. Hahn RT, Pibarot P, Stewart WJ, et al. Comparison of transcatheter and surgical aortic valve replacement in severe aortic stenosis: a longitudinal study of echocardiography parameters in cohort A of the PARTNER Trial (Placement of Aortic Transcatheter Valves). J Am Coll Cardiol. Jun 25 2013;61(25):2514-21. [View Abstract]
  33. Adams DH, Popma JJ, Reardon MJ, Yakubov SJ, Coselli JS, Deeb GM, et al. Transcatheter Aortic-Valve Replacement with a Self-Expanding Prosthesis. N Engl J Med. Mar 29 2014;[View Abstract]
  34. Wood S. CoreValve Beats High-Risk Surgery for AV Stenosis in Pivotal Trial. Medscape [serial online]. Available at http://www.medscape.com/viewarticle/822728. Accessed April 8, 2014.
  35. Neale T. FDA OKs expanded use for CoreValve. Medpage Today [serial online]. June 12, 2014;Accessed June 16, 2014. Available at http://www.medpagetoday.com/Cardiology/PCI/46297?xid=nl_mpt_DHE_2014-06-13&utm_content=&utm_medium
  36. Medtronic, Inc. Medtronic CoreValve System receives FDA approval for patients at high risk for surgery [press release]. June 12, 2014. Available at http://newsroom.medtronic.com/phoenix.zhtml?c=251324&p=irol-newsArticle&ID=1939539&highlight=. Accessed June 16, 2014.
  37. [Guideline] Chobanian AV, Bakris GL, Black HR, Cushman WC, Green LA, Izzo JL Jr, et al. The Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure: the JNC 7 report. JAMA. May 21 2003;289(19):2560-72. [View Abstract]
  38. [Guideline] Nishimura RA, Carabello BA, Faxon DP, et al. ACC/AHA 2008 Guideline Update on Valvular Heart Disease: Focused Update on Infective Endocarditis: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Journal of the American College of Cardiology. August 2008;52:676-85. [View Abstract]
  39. Moura LM, Ramos SF, Zamorano JL, Barros IM, Azevedo LF, Rocha-Gonçalves F, et al. Rosuvastatin affecting aortic valve endothelium to slow the progression of aortic stenosis. J Am Coll Cardiol. Feb 6 2007;49(5):554-61. [View Abstract]
  40. Chan KL, Teo K, Dumesnil JG, Ni A, Tam J. Effect of Lipid lowering with rosuvastatin on progression of aortic stenosis: results of the aortic stenosis progression observation: measuring effects of rosuvastatin (ASTRONOMER) trial. Circulation. Jan 19 2010;121(2):306-14. [View Abstract]
  41. Rossebo AB, Pedersen TR, Boman K, et al. Intensive lipid lowering with simvastatin and ezetimibe in aortic stenosis. N Engl J Med. Sep 25 2008;359(13):1343-56. [View Abstract]
  42. Cowell SJ, Newby DE, Prescott RJ, et al. A randomized trial of intensive lipid-lowering therapy in calcific aortic stenosis. N Engl J Med. Jun 9 2005;352(23):2389-97. [View Abstract]
  43. [Guideline] Wann LS, Curtis AB, Ellenbogen KA, et al. 2011 ACCF/AHA/HRS Focused Update on the Management of Patients With Atrial Fibrillation (Update on Dabigatran): A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. Mar 15 2011;123(10):1144-50. [View Abstract]
  44. [Best Evidence] Bagur R, Webb JG, Nietlispach F, Dumont E, De Larochellière R, Doyle D, et al. Acute kidney injury following transcatheter aortic valve implantation: predictive factors, prognostic value, and comparison with surgical aortic valve replacement. Eur Heart J. Apr 2010;31(7):865-74. [View Abstract]
  45. Eltchaninoff H, Prat A, Gilard M et al,. Transcatheter aortic valve implantation: early results of the FRANCE (FRench Aortic National CoreValve and Edwards) registry. Eur Heart Jl. 2011;32:191–197.
  46. FDA approval expands access to artificial heart valve for inoperable patients [press release]. Food and Drug Administration; September 23, 2013.
  47. Lefevre T, Kappetein AP, Wolner E, et al. One year follow-up of the multi-centre European PARTNER transcatheter heart valve study. Eur Heart Jl. 2011;32:148–157.
  48. Leon MB, Smith CR, Mack M, et al. Transcatheter aortic-valve implantation for aortic stenosis in patients who cannot undergo surgery. N Engl J Med. Oct 21 2010;363(17):1597-607. [View Abstract]
  49. Prasad Y, Bhalodkar NC. Aortic sclerosis--a marker of coronary atherosclerosis. Clin Cardiol. Dec 2004;27(12):671-3. [View Abstract]
  50. [Best Evidence] Rosenhek R, Zilberszac R, Schemper M, Czerny M, Mundigler G, Graf S, et al. Natural history of very severe aortic stenosis. Circulation. Jan 5 2010;121(1):151-6. [View Abstract]
  51. Tamburino C, Capodanno D, Ramondo A, Petronio AS, Ettori F, Santoro G, et al. Incidence and predictors of early and late mortality after transcatheter aortic valve implantation in 663 patients with severe aortic stenosis. Circulation. Jan 25 2011;123(3):299-308. [View Abstract]
  52. Wood S. Expanded FDA indication for Sapien valve. Medscape Medical News [serial online]. September 24, 2013;Accessed October 1, 2013. Available at http://www.medscape.com/viewarticle/811544
  53. Zahn R, Gerckens U, EGrube E et al. Transcatheter aortic valve implantation: first results from a multi-centre real-world registry. Eur Heart Jl. 2011;32:198–204.
  54. Zajarias A, Cribier AG. Outcomes and safety of percutaneous aortic valve replacement. J Am Coll Cardiol. May 19 2009;53(20):1829-36. [View Abstract]

Calcific aortic stenosis (parasternal long-axis and short-axis views).

Calcific aortic stenosis (parasternal long-axis and short-axis views).

Calcific aortic stenosis (parasternal long-axis and short-axis views).

Stenotic aortic valve (macroscopic appearance).

Age < 70 years (n=324) Age >70 years (n=322)
Bicuspid AV (50%)

Postinflammatory (25%)

Degenerative (18%)

Unicommissural (3%)

Hypoplastic (2%)

Indeterminate (2%)

Degenerative (48%)

Bicuspid (27%)

Postinflammatory (23%)

Hypoplastic (2%)

Indication Class
Diagnosis and assessment of severity of aortic stenosisI
Assessment of LV size, function, and/or hemodynamicsI
Reevaluation of patients with known aortic stenosis with changing symptoms or signsI
Assessment of changes in hemodynamic severity and ventricular function in patients with known aortic stenosis during pregnancyI
Reevaluation of asymptomatic patients with severe aortic stenosisI
Reevaluation of asymptomatic patients with mild to moderate aortic stenosis and evidence of LV dysfunction or hypertrophyIIa
Routine reevaluation of asymptomatic adult patients with mild aortic stenosis who have stable physical signs and normal LV size and function III
Severity Mean gradient (mm Hg) Aortic valve area (cm2)
Mild< 25>1.5
Moderate25-401-1.5
Severe>40< 1

(or < 0.5 cm2/m2 body surface area)

Critical>80< 0.5
Indication Class
Coronary angiography before aortic valve replacement in patients at risk for coronary artery diseaseI
Assessment of severity of aortic stenosis in symptomatic patients when aortic valve replacement is planned or when noninvasive tests are inconclusive or a discrepancy exists in the clinical findings regarding the severity of aortic stenosis or the need for surgery I
Coronary angiography before aortic valve replacement in patients for whom a pulmonary autograft (Ross procedure) is contemplated and the origin of the coronary arteries was not identified by noninvasive tests I
With infusion of dobutamine, can be useful for evaluation of patients with low-flow/low-gradient aortic stenosis and LV dysfunctionIIa
Not recommended for hemodynamic measurements for assessment of aortic stenosis severity when noninvasive techniques are adequate and concord with clinical findings III
Not recommended for hemodynamic measurements for assessment of LV function and aortic stenosis severity in asymptomatic patientsIII
Indication Class
Symptomatic patients with severe aortic stenosisI
Patients with severe aortic stenosis undergoing coronary artery bypass surgeryI
Patients with severe aortic stenosis undergoing surgery on the aorta or other heart valvesI
Patients with severe aortic stenosis and LV systolic dysfunction (ejection fraction < 0.50)I
Patients with moderate aortic stenosis undergoing coronary artery bypass surgery or surgery on the aorta or other heart valvesIIa
Patients with mild aortic stenosis undergoing coronary artery bypass surgery when there is evidence that progression may be rapid, such as moderate-to-severe valve calcificationIIb
Asymptomatic patients with severe aortic stenosis and abnormal response to exercise (eg, hypotension)IIb
Asymptomatic patients with severe aortic stenosis and a high likelihood of rapid progression (based on age, calcification, and coronary artery disease) or if surgery might be delayed at the time of symptom onsetIIb
Asymptomatic patients with extremely severe aortic stenosis (valve area less than 0.6 cm2, mean gradient greater than 60 mm Hg, and jet velocity greater than 5 m per second) if the patient’s expected operative mortality is 1% or lessIIb
AVR is not useful for prevention of sudden death in asymptomatic patients with none of the findings listed under asymptomatic patients with severe aortic stenosisIII