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Aortic Stenosis

By

Guy P. Armstrong

, MD, North Shore Hospital, Auckland

Last full review/revision Aug 2021
Topic Resources

Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. Causes include a congenital bicuspid valve, idiopathic degenerative sclerosis with calcification, and rheumatic fever. Untreated AS progresses to become symptomatic with one or more of the classic triad of syncope, angina, and exertional dyspnea; heart failure and arrhythmias may develop. A crescendo-decrescendo ejection murmur is characteristic. Diagnosis is by physical examination and echocardiography. Asymptomatic AS in adults usually requires no treatment. Once symptoms develop, surgical or percutaneous valve replacement is required. For severe or symptomatic AS in children, balloon valvotomy is effective.

Etiology of Aortic Stenosis

In older patients, the most common precursor to aortic stenosis is

  • Aortic sclerosis

Aortic sclerosis is a degenerative aortic valve disease with thickening of aortic valve structures by fibrosis and calcification initially without causing significant obstruction. Over years, aortic sclerosis progresses to stenosis in as many as 15% of patients. Aortic sclerosis resembles atherosclerosis Atherosclerosis Atherosclerosis is characterized by patchy intimal plaques (atheromas) that encroach on the lumen of medium-sized and large arteries; the plaques contain lipids, inflammatory cells, smooth muscle... read more Atherosclerosis , with deposition of lipoproteins and inflammation and calcification of the valves; risk factors Risk Factors Atherosclerosis is characterized by patchy intimal plaques (atheromas) that encroach on the lumen of medium-sized and large arteries; the plaques contain lipids, inflammatory cells, smooth muscle... read more Risk Factors are similar: lipoprotein (a) is implicated in the pathogenesis of both aortic stenosis and atherosclerosis. Elevated lipoprotein (a) levels also predict faster hemodynamic progression of AS. Patients with psoriasis Psoriasis Psoriasis is an inflammatory disease that manifests most commonly as well-circumscribed, erythematous papules and plaques covered with silvery scales. Multiple factors contribute, including... read more Psoriasis are at increased risk for atherosclerosis, and more recently, psoriasis has been tied to an increased risk of aortic stenosis.

In patients < 70 years, the most common cause of aortic stenosis is

In medically underserved countries, the most common cause of aortic stenosis in all age groups is

Supravalvular AS caused by a discrete congenital membrane or hypoplastic constriction just above the sinuses of Valsalva is uncommon. A sporadic form of supravalvular AS is associated with a characteristic facial appearance (high and broad forehead, hypertelorism, strabismus, upturned nose, long philtrum, wide mouth, dental abnormalities, puffy cheeks, micrognathia, low-set ears). When associated with idiopathic hypercalcemia of infancy Neonatal Hypercalcemia Hypercalcemia is total serum calcium > 12 mg/dL (> 3 mmol/L) or ionized calcium > 6 mg/dL (> 1.5 mmol/L). The most common cause is iatrogenic. Gastrointestinal signs may occur (eg, anorexia... read more , this form is known as Williams syndrome.

Subvalvular AS caused by a congenital membrane or fibrous ring just beneath the aortic valve is uncommon.

Pathophysiology of Aortic Stenosis

The increased pressure load imposed by aortic stenosis results in compensatory hypertrophy of the left ventricle (LV) without cavity enlargement (concentric hypertrophy). With time, the ventricle can no longer compensate, causing secondary LV cavity enlargement, reduced ejection fraction (EF), decreased cardiac output, and a misleadingly low gradient across the aortic valve (low-gradient severe AS). Patients with other disorders that also cause LV enlargement and reduced EF (eg, myocardial infarction, intrinsic cardiomyopathy) may generate insufficient flow to fully open a sclerotic valve and have an apparently small valve area even when their AS is not particularly severe (pseudosevere AS). Pseudosevere AS must be differentiated from low-gradient severe AS because only patients with low-gradient severe AS benefit from valve replacement.

Elevated shear stress across the stenosed aortic valve degrades von Willebrand factor multimers. The resulting coagulopathy may cause gastrointestinal bleeding in patients with angiodysplasia (Heyde syndrome).

Symptoms and Signs of Aortic Stenosis

Congenital aortic stenosis is usually asymptomatic until age 10 or 20 years, when symptoms develop insidiously. In all forms, progressive untreated aortic stenosis ultimately results in exertional syncope, angina, and dyspnea (SAD triad). Other symptoms and signs may include those of heart failure Heart Failure (HF) Heart failure (HF) is a syndrome of ventricular dysfunction. Left ventricular failure causes shortness of breath and fatigue, and right ventricular failure causes peripheral and abdominal fluid... read more Heart Failure (HF) and arrhythmias Overview of Arrhythmias The normal heart beats in a regular, coordinated way because electrical impulses generated and spread by myocytes with unique electrical properties trigger a sequence of organized myocardial... read more Overview of Arrhythmias , including ventricular fibrillation leading to sudden death.

Exertional syncope occurs because cardiac output cannot increase enough to meet the demands of physical activity. Nonexertional syncope may result from altered baroreceptor responses or ventricular tachycardia Ventricular Tachycardia (VT) Ventricular tachycardia is ≥ 3 consecutive ventricular beats at a rate ≥ 120 beats/minute. Symptoms depend on duration and vary from none to palpitations to hemodynamic collapse and death. Diagnosis... read more . Exertional angina pectoris affects about two thirds of patients; about half have significant coronary artery atherosclerosis, and half have normal coronary arteries but have ischemia induced by LV hypertrophy and altered coronary flow dynamics.

There are no visible signs of aortic stenosis. Palpable signs include carotid and peripheral pulses that are reduced in amplitude and slow rising (pulsus parvus et tardus) and an apical impulse that is sustained (thrusts with the 1st heart sound [S1] and relaxes with the 2nd heart sound [S2]) because of left ventricular hypertrophy. The LV impulse may become displaced when systolic dysfunction develops. A palpable 4th heart sound (S4), felt best at the apex, and a systolic thrill, corresponding with the murmur of AS and felt best at the left upper sternal border, are occasionally present in severe cases. Systolic blood pressure (BP) may be high even when AS is severe but ultimately falls when the LV fails.

On auscultation, S1 is normal and S2 is single because aortic valve closing is delayed and merges with the pulmonic (P2) component of S2. The aortic component may also be soft. Paradoxical splitting of S2 may be heard. A normally split S2 is the only physical finding that reliably excludes severe AS. An S4 may be audible. An ejection click may also be audible early after S1 in patients with congenital bicuspid AS when valve leaflets are stiff but not completely immobile. The click does not change with dynamic maneuvers.

The hallmark finding is a crescendo-decrescendo ejection murmur, heard best with the diaphragm of the stethoscope at the right and left upper sternal border when a patient who is sitting upright leans forward. The murmur typically radiates to the right clavicle and both carotid arteries (left often louder than right) and has a harsh or grating quality. But in older patients, vibration of the unfused cusps of calcified aortic valve leaflets may transmit a louder, more high-pitched, “cooing” or musical sound to the cardiac apex, with softening or absence of the murmur parasternally (Gallavardin phenomenon), thereby mimicking mitral regurgitation. The murmur is soft when stenosis is less severe, grows louder as stenosis progresses, and becomes longer and peaks in volume later in systole (ie, crescendo phase becomes longer and decrescendo phase becomes shorter) as stenosis becomes more severe. As LV contractility decreases in critical AS, the murmur becomes softer and shorter. The intensity of the murmur may therefore be misleading in these circumstances.

The murmur of aortic stenosis typically increases with maneuvers that increase LV volume and contractility (eg, leg-raising, squatting, Valsalva release, after a ventricular premature beat) and decreases with maneuvers that decrease LV volume (Valsalva maneuver) or increase afterload (isometric handgrip). These dynamic maneuvers have the opposite effect on the murmur of hypertrophic cardiomyopathy, which can otherwise resemble that of AS. The murmur of mitral regurgitation due to prolapse of the posterior leaflet may also mimic AS.

Diagnosis of Aortic Stenosis

  • Echocardiography

Diagnosis of aortic stenosis is suspected clinically and confirmed by echocardiography Echocardiography This photo shows a patient having echocardiography. This image shows all 4 cardiac chambers and the tricupsid and mitral valves. Echocardiography uses ultrasound waves to produce an image of... read more Echocardiography . Two-dimensional transthoracic echocardiography is used to identify a stenotic aortic valve and possible causes, to quantify LV hypertrophy and degree of systolic dysfunction, and to detect coexisting valvular heart disorders (aortic regurgitation, mitral valve disorders) and complications (eg, endocarditis). Doppler echocardiography is used to quantify degree of stenosis by measuring jet velocity, transvalvular systolic pressure gradient, and aortic valve area.

Severity of aortic stenosis is characterized echocardiographically as

  • Mild: Peak aortic jet velocity 2 to 2.9 m/second or mean gradient < 20 mm Hg

  • Moderate: Peak aortic jet velocity 3 to 4 m/second or mean gradient 20 to 40 mm Hg

  • Severe: Peak aortic jet velocity > 4 m/second or mean gradient > 40 mm Hg

  • Very severe: Peak aortic jet velocity > 5 m/second or mean gradient > 60 mm Hg

Clinical judgment and critical review of the data are used to resolve any discordance among these parameters (eg, moderate valve area but severe mean gradient). Measurement of aortic valve area is least accurate when LV volume or systolic function is reduced, or if systemic hypertension is present.

The gradient may be overestimated when aortic regurgitation Aortic Regurgitation Aortic regurgitation (AR) is incompetency of the aortic valve causing backflow from the aorta into the left ventricle during diastole. Causes include valvular degeneration and aortic root dilation... read more Aortic Regurgitation is present. The gradient may under-represent severity when the stroke volume is low, eg, in patients with systemic hypertension or LV systolic dysfunction (low-gradient AS with reduced EF) or a small, hypertrophied LV (low-gradient AS with normal EF—see table Types of Severe Aortic Stenosis Types of Severe Aortic Stenosis Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. Causes include a congenital bicuspid valve, idiopathic... read more Types of Severe Aortic Stenosis ). Sometimes LV systolic dysfunction results in low ventricular pressure that is inadequate to open nonstenotic valve leaflets, causing echocardiographic appearance of low valve area in the absence of stenosis (pseudostenosis). Differentiation of pseudostenosis from low-gradient AS can be aided by calculation of the ratio of outflow tract to aortic velocity (Doppler velocity index, DVI). A DVI value < 0.25 suggests true severe stenosis. Assessment of the degree of valve calcification by CT can help determine the severity of AS. Severe AS is likely when the valvular calcium score is > 2000 (men) and > 1300 (women). It is very likely when the calcium score is > 3000 (men) and > 1600 (women). Low-dose dobutamine stress echocardiography distinguishes low-gradient AS from pseudostenosis.

Table
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Cardiac catheterization Cardiac Catheterization Cardiac catheterization is the passage of a catheter through peripheral arteries or veins into cardiac chambers, the pulmonary artery, and coronary arteries and veins. Cardiac catheterization... read more Cardiac Catheterization is necessary prior to intervention to determine whether coronary artery disease (CAD) is the cause of angina and, occasionally, to resolve inconsistency between clinical and echocardiographic findings.

An ECG and chest x-ray are obtained.

Chest x-ray findings may include calcification of the aortic cusps (seen on the lateral projection or on fluoroscopy) and evidence of heart failure. Heart size may be normal or only mildly enlarged.

In asymptomatic patients with severe aortic stenosis, closely supervised exercise ECG testing Stress Testing In stress testing, the heart is monitored by electrocardiography (ECG) and often imaging studies during an induced episode of increased cardiac demand so that ischemic areas potentially at risk... read more Stress Testing is recommended in an attempt to elicit symptoms of angina, dyspnea, or hypotension—any of these symptoms, when due to the AS, is an indication for intervention. Failure to normally increase BP and development of ST segment depression are less predictive of adverse prognosis. Exercise testing is contraindicated in symptomatic patients.

Diagnosis reference

  • 1. Otto CM, Nishimura RA, Bonow RO, et al: 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation 143(5):e35–e71, 2021. doi: 10.1161/CIR.0000000000000932

Prognosis for of Aortic Stenosis

Aortic stenosis progresses faster as severity increases, but the wide variability in progression rates requires regular surveillance, particularly in sedentary older patients. In such patients, flow may become significantly compromised without triggering symptoms.

Asymptomatic patients with severe AS and normal systolic function should be reevaluated every 6 months because 3 to 6% will develop symptoms or LVEF impairment every year. The risk of surgery outweighs the survival benefit in asymptomatic patients, but with the onset of symptoms the mean survival plummets to 2 to 3 years, and prompt valve replacement is indicated to relieve symptoms and improve survival. Risk of surgery increases for patients who require simultaneous coronary artery bypass graft (CABG) and for those with depressed systolic LV function.

In patients with severe AS, about 50% of deaths occur suddenly, and these patients should be advised to limit physical exertion.

Treatment of Aortic Stenosis

  • Sometimes aortic valve replacement

Nothing has yet been proved to slow the progression of aortic stenosis. In randomized trials, statin therapy has been ineffective.

Drugs that can cause hypotension (eg, nitrates) should be used cautiously, although nitroprusside has been used as a temporizing measure to reduce afterload in patients with decompensated heart failure in the hours before valve replacement. Patients who develop heart failure but are too high risk for valve intervention benefit from cautious treatment with digoxin, diuretics, and angiotensin-converting enzyme (ACE) inhibitors.

Timing of intervention

The benefits of intervention do not outweigh the risks until patients develop symptoms and/or meet certain echocardiographic criteria. Thus, patients should have periodic clinical evaluations, including echocardiography and sometimes exercise testing, to determine the optimal time for valve replacement. Valve replacement is recommended when aortic stenosis is severe and there is any one of the following:

  • Symptoms

  • Exercise testing causing symptoms or showing reduced effort tolerance or a fall in blood pressure ≥ 10 mm Hg below baseline)

  • LV EF < 50%

If surgical risk is low, then surgery may be considered if there is any one of:

  • Very severe AS (aortic velocity > 5 m/second)

  • Brain (B-type) natriuretic peptide (BNP) is > 3 times normal

  • Severe pulmonary hypertension without other explanation

  • Decline in EF to < 60%

  • Severe valve calcification with rapid progression of stenosis (reduction in aortic valve area ≥ 0.3 m/second/year)

When cardiac surgery is being done for other reasons, concomitant aortic valve surgery is indicated regardless of symptoms if the AS is moderate or greater.

Choice of intervention

Balloon valvotomy is used primarily in children and very young adults with congenital AS.

In older patients who are not candidates for surgery, balloon valvuloplasty has been used as a bridge to valve replacement but this procedure has a high complication rate and provides only temporary relief. It is being replaced by transcatheter valve implantation, which can be done with similar procedural risk, even in sick, high-risk patients.

Surgical aortic valve replacement (SAVR) usually involves replacement with a mechanical or bioprosthetic valve, but in younger patients, the patient’s own pulmonic valve can be used, with a bioprosthesis then used to replace the pulmonic valve (Ross procedure).

Transcatheter (percutaneous) aortic valve implantation (TAVI) (sometimes called transcatheter aortic valve replacement, or TAVR) is a less invasive method of aortic valve replacement for which the indications continue to expand.

The choice is between the up-front risk and morbidity of SAVR versus the unknown durability of TAVI valves beyond 5 years. Guidelines to inform shared decision-making suggest SAVR for patients < 65 years; TAVI is preferred for patients suitable for the transfemoral approach who are > 80 years or who have a life expectancy of < 10 years. In patients aged 65 to 80 years who are suitable for transfemoral TAVI, the decision regarding SAVR or TAVI is determined based on individual patient characteristics (1 Treatment reference Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. Causes include a congenital bicuspid valve, idiopathic... read more Treatment reference ). If the patient’s arteries are not suited to the transfemoral route, then SAVR is preferred as long as surgical risk is not prohibitive. In patients with a life expectancy of < 1 year even with a successful procedure, intervention is not recommended. Compared with SAVR, transfemoral TAVI has lower short-term mortality, and less risk of stroke, major bleeding, and atrial fibrillation. TAVI also requires a shorter hospital stay, causes less pain, and permits more rapid return to activity; however, it also increases vascular complications and paravalvular regurgitation and increases the need for permanent pacemaker implantation and early repeat valve intervention.

Preoperative evaluation for CAD is indicated so that CABG and valve replacement, if indicated, can be done during the same procedure. An aortic bioprosthetic valve requires anticoagulation for 3 to 6 months postoperatively, but a mechanical valve requires lifetime anticoagulation using warfarin. Newer direct-acting oral anticoagulants (DOAC) are ineffective and should not be used (see also Anticoagulation for patients with a prosthetic cardiac valve Anticoagulation for patients with a prosthetic cardiac valve Any heart valve can become stenotic or insufficient (also termed regurgitant or incompetent), causing hemodynamic changes long before symptoms. Most often, valvular stenosis or insufficiency... read more ).

Treatment reference

  • 1. Otto CM, Nishimura RA, Bonow RO, et al: 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation 143(5):e35–e71, 2021. doi: 10.1161/CIR.0000000000000932

Key Points

  • The most common cause of aortic stenosis (AS) in patients < 70 years is bicuspid aortic valve; aortic sclerosis is the most common precursor in older patients.

  • Untreated AS ultimately results in exertional syncope, angina, and dyspnea; sudden death may occur.

  • Typical heart sounds are a crescendo-decrescendo ejection murmur that increases with maneuvers that increase left ventricular (LV) volume and contractility (eg, leg-raising, squatting, Valsalva release) and decreases with maneuvers that decrease LV volume (Valsalva maneuver) or increase afterload (isometric handgrip).

  • Nitrates may cause dangerous hypotension and should be used with caution for angina in patients with AS.

  • Exercise testing is now recognized as particularly useful in risk stratification and timing of surgery for patients without clear symptoms.

  • Replacement is indicated once symptoms begin or LV dysfunction occurs.

  • Surgical or transcatheter aortic valve replacement are options for many patients, but long-term safety data is still lacking for transcatheter aortic valve replacement (especially in low-risk patients).

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Aortic Stenosis
Aortic stenosis (AS) occurs when the aortic valve narrows, obstructing blood flow from the left ventricle to the ascending aorta during systole. There are several different causes of AS, and the causes differ among age groups. However, in low and middle income countries, which of the following is the most common cause of AS in all age groups?  
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