Aortic Stenosis Flashcards

1
Q

Summarise aortic stenosis

A

Obstruction of blood flow across the aortic valve due to aortic calcification.

Presentation includes shortness of breath with exertion, angina, or syncope.

Characteristic murmur is systolic, mid-to-late peaking with a crescendo-decrescendo pattern, and radiates to the carotids.

Doppler echo is essential to the diagnosis and will show a pressure gradient across the stenotic aortic valve.

Surgical aortic valve replacement was the only effective therapy for aortic stenosis for over 50 years. However, with the advent of transcatheter valve therapies, patients and physicians have more options.

Following valve replacement, patients are subject to the complications of prosthetic valves.

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2
Q

Define aortic stenosis

A

Aortic stenosis (AS) represents obstruction of blood flow across the aortic valve due to pathological narrowing. It is a progressive disease that presents after a long subclinical period with symptoms of decreased exercise capacity, exertional chest pain (angina), syncope, and heart failure.

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3
Q

Describe the epidemiology of aortic stenosis

A

AS is the most common valvular disease in the US and Europe and is the second most frequent cause for cardiac surgery. It is largely a disease of older people, and patients typically present in the seventh or eighth decade of life. Although the prevalence of AS is only 0.2% in adults aged 50 to 59 years, estimates increase to 1.3% in patients aged 60 to 69 years, and up to 9.8% in patients aged 80 to 89 years.[1] Overall, AS is present in 2.6% of adults older than 75 years of age.[2] It is preceded by aortic sclerosis (defined as aortic valve thickening without flow limitation), often suspected by the presence of an early-peaking, systolic ejection murmur, and confirmed by echocardiography. Nearly 25% of people ≥65 years have aortic sclerosis, and nearly 17% of people with aortic sclerosis will progress to AS in their lifetime.[3] The average time from diagnosis of aortic sclerosis to the development of moderate and severe AS is 6 and 8 years, respectively

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4
Q

Which patients are at a particular risk of developing aortic stenosis at an earlier age

A

Patients with bicuspid valves and AS present two decades earlier on average than patients with trileaflet valves.[4] Congenital bicuspid aortic valves affect 0.9% to 1.36% of the general population with a 2:1 male:female predominance.[5] Overall, about half of all aortic valve replacements are performed for congenitally malformed valves. A study of a large series of patients undergoing aortic valve replacement for AS found that in patients <50 years old, one third had unicuspid valves, and two-thirds had bicuspid valves. Among patients aged 50 to 70 years, two-thirds had bicuspid valves and one third had trileaflet valves. For patients >70 years old, 60% had trileaflet valves, and 40% had bicuspid valves

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5
Q

Describe the aetiology of aortic stenosis

A

Calcification of normal trileaflet valves is the most common cause of AS in adults and accounts for as many as 80% of cases in the US and Europe.[7] Calcific aortic disease represents a spectrum ranging from aortic sclerosis (defined as leaflet thickening without obstruction) to severe AS. Several risk factors have been associated with aortic sclerosis including smoking, hypertension, diabetes, low-density lipoprotein (LDL)-cholesterol, and elevated C-reactive protein. Retrospective studies have shown that high LDL-cholesterol levels and smoking are associated with progression to AS, but causality has not been confirmed

Congenitally bicuspid valves account for the majority of the remainder of cases. Patients with coarctation of the aorta and Turner’s syndrome have a higher incidence of bicuspid valves.

Rheumatic heart disease has historically been an important cause of AS, but due to improvements in treatment, it is now uncommon in industrialised countries. Rheumatic heart disease remains prevalent in developing nations.

Other circumstances including connective tissue diseases, radiotherapy, and hyperlipoproteinaemia syndromes can cause AS, but these are unusual consequences of rare conditions.

Chronic kidney disease is associated with abnormal calcium homeostasis, and it has been shown that AS progresses faster in patients with this condition.

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6
Q

Describe the process of aortic calcification

A

Aortic calcification is no longer thought to reflect age-related wear and tear, and is recognised to be an active process. The valvular endocardium is damaged as the result of abnormal blood flow across the valve. Endocardial injury initiates an inflammatory process similar to atherosclerosis and ultimately leads to deposition of calcium on the valve. Calcification occurs slowly and is subclinical until the disease is fairly advanced. Progressive calcium deposition limits aortic leaflet mobility and eventually produces stenosis.

Unicuspid and bicuspid valves experience abnormal shear and mechanical stresses from birth. Therefore, the pathological processes and resultant stenosis occur earlier than in trileaflet valves.

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7
Q

What causes the calcification in rheumatic disease

A

In rheumatic disease, an autoimmune inflammatory reaction is triggered by prior Streptococcus infection that targets the valvular endothelium, leading to inflammation and eventually calcification

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8
Q

Describe LV remodelling in response to the aortic stenosis

A

Long-standing pressure overload leads to the development of left ventricular hypertrophy (LVH). This adaptive response permits the ventricle to maintain a normal wall stress (afterload) despite the pressure overload produced by stenosis. As the stenosis worsens, the adaptive mechanism fails and left ventricular wall stress increases. Systolic function declines as wall stress increases, and eventually the heart fails.

LVH is a contributing factor to many of the symptoms seen in AS. The consequence of concentric LVH is a smaller, less compliant chamber. Thus, left ventricle end-diastolic pressure is increased, especially during periods of increased cardiac output (e.g., exercise), leading to the sensation of dyspnoea. Furthermore, in LVH, myocardial oxygen demand is greater due to increased left ventricular mass, while coronary blood flow may be reduced through a variety of mechanisms. Thus, even patients who lack coronary atherosclerotic disease may develop symptoms of anginal chest pain.

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9
Q

Describe a typical case history of aortic stenosis

A

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

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10
Q

Describe some other presentations of aortic stenosis

A

Other presentations
AS is a progressive disease that presents after a long subclinical period with symptoms of chest pain, syncope, and heart failure. While the most common complaint is dyspnoea with exertion, patients also frequently note syncope or chest pain that may be identical to that caused by coronary artery disease. Many cases of AS are diagnosed during the subclinical phase while a murmur noted on physical examination is being investigated. Even with severe AS, patients may be truly asymptomatic. A careful history is important to determine if the patient has altered his or her habits in response to slowly worsening stenosis.

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11
Q

Summarise the clinical evaluation of aortic stenosis

A

Many cases of AS are diagnosed during the subclinical phase while a murmur, noted on physical examination, is being investigated. Even patients with severe AS may be truly asymptomatic. A careful history is important to determine if the patient has altered his or her habits in response to slowly worsening stenosis.

Complaints of decreased exercise tolerance, shortness of breath on exertion, exertional chest pain (angina), syncope, or near syncope, and heart failure symptoms should prompt consideration of AS. Patients have an increased risk of bleeding and may complain of epistaxis or bruising. They are also more likely to develop chronic gastrointestinal bleeding that is associated with angiodysplasia. This may be due to turbulent flow across the stenotic valve producing an acquired von Willebrand deficiency.

The physical examination is the most important screening tool for valvular heart disease. A complete cardiac examination including precordial palpation, auscultation with attention to murmurs and the aortic closure sound, and evaluation of arterial and venous pulsation is essential in generating clinical suspicion for AS.

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12
Q

Describe the general grading of murmurs

A

Murmurs are generally graded on a scale of 1 to 6:

Grade 1: murmur is faint and heard only with effort
Grade 2: murmur is faint but easily detected
Grade 3: murmur is loud
Grade 4: murmur is very loud and associated with a palpable thrill
Grade 5: murmur is so loud that it can be heard with only the edge of the stethoscope
Grade 6: murmur is extremely loud and heard even when the stethoscope is no longer in contact with the patient.

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13
Q

Describe the murmur in aortic stenosis

A

The typical murmur of AS is a systolic murmur ≥3/6, with a diamond-shaped crescendo-decrescendo pattern that peaks in mid-systole and radiates to the carotid arteries. The murmur is generally loudest at the right upper sternal border and terminates with S2 and the end of systole. As the severity of stenosis worsens, the murmur peaks later in systole and may be accompanied by a palpable thrill. The aortic heart sound (A2) is delayed as the systolic ejection period becomes prolonged. In severe stenosis, paradoxical splitting of the second heart sound may be noted such that in expiration, the pulmonic sound (P2) is heard before A2. The intensity of the second heart sound tends to be diminished as the severity of stenosis increases and leaflet mobility is reduced.

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14
Q

Describe some uncommon murmur characteristics in aortic stenosis

A

Uncommon findings include the presence of a holosystolic murmur at the apex (Gallavardin’s phenomenon), mimicking the murmur of mitral regurgitation.

A delayed and diminished carotid upstroke (carotid parvus et tardus) may occur with severe AS, although this finding is often difficult to distinguish in older patients. In these circumstances, palpation of the brachial artery may reveal this finding.

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15
Q

Describe the ECG in aortic stenosis

A

An ECG is indicated in the initial work-up of all patients and is abnormal in >90% of patients with AS, with the most common abnormality being left ventricular hypertrophy (LVH) due to pressure overload.[19] Evidence of LVH and absent Q waves helps distinguish AS from other conditions such as aortic sclerosis with ischaemic heart disease. Patients with AS often have conduction disease manifesting as atrioventricular (AV) block, hemiblock, or bundle branch block.

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16
Q

Describe the use of echocardiography

A

Echocardiography is the test of choice in the evaluation of suspected AS and for the evaluation of murmurs detected on physical examination. The American College of Cardiology/American Heart Association (ACC/AHA) recommends transthoracic echocardiography (TTE) when there is an unexplained systolic murmur, a single second heart sound, a history of a bicuspid aortic valve, or symptoms that might be due to AS.[20]

Although murmur intensity does not correlate well with the haemodynamic significance of the associated lesion, grade 3 murmurs are generally thought to reflect more significant lesions and thus warrant further evaluation with TTE. In practice, most patients with suspected cardiac disease and a murmur on examination should have an echocardiogram.

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17
Q

Describe Doppler transthoracicechocardiogram

A

Doppler TTE can reliably and accurately detect the presence of a pressure gradient across the aortic valve. It can also assess left ventricle function and the presence of hypertrophy. It is essential for the diagnosis of AS and for serial evaluation once the diagnosis has been established. Measurements taken during echo examination are used to grade the severity of AS

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18
Q

Describe trans oesophageal echocardiography

A

Transoesophageal echocardiography (TEE) provides alternative detailed views of the aortic valve apparatus, and is frequently used in patients undergoing valve surgery or transcatheter aortic valve replacement (TAVR). However, the test is invasive and is not recommended in the routine evaluation of AS given the efficacy and safety of Doppler TTE.

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19
Q

Describe cardiac MRI

A

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

20
Q

Describe ECG exercise stress testing

A

Symptomatic patients should not have exercise testing and should be referred for valve replacement. For those without symptoms, exercise testing may provide clinically important information.[21] Nearly 40% of patients with asymptomatic AS will develop symptoms with exercise testing.[22] Exercise echocardiography may add additional diagnostic and prognostic information.[23][24] In a group of patients with asymptomatic severe AS, a positive exercise stress test (defined as the onset of symptoms, ST changes, abnormal blood pressure response, or complex ventricular arrhythmia) was highly predictive of the onset of symptoms or the need for surgery. At 24 months, only 19% with a positive exercise test remained symptom-free or without valve replacement compared with 85% of those with a negative test.[25] Subsequent work found that the development of symptoms on exercise testing was the strongest predictor for the onset of spontaneous symptoms, especially among patients <70 years old in whom symptoms of fatigue and breathlessness are more specific than in older patients.

21
Q

Describe the dobutamine stress echo

A

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

22
Q

Describe cardiac catheterisation

A

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

23
Q

Describe the key diagnostic features in the history and the exam

A

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

dyspnoea
Shortness of breath on exertion is the most common presenting complaint.

Dyspnoea occurs at a prevalence of 60%.[27]

chest pain
Patients frequently develop exertional chest pain (angina). Symptoms may occur even in the absence of coronary atherosclerotic disease.

Chest pain occurs at a prevalence of 50%.[27]

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

The prevalence of syncope is 40%.[27]

ejection systolic murmur
A systolic murmur ≥3/6 is present with a crescendo-decrescendo pattern that peaks in mid-systole and radiates to the carotid.
The murmur is loudest at the right upper sternal border.

S2 diminished and single
With progression of AS, the aortic component of the second heart sound becomes soft or absent due to decreased mobility of the aortic valve leaflets.

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

24
Q

Describe an uncommon diagnostic feature

A

carotid parvus et tardus
The carotid upstroke is frequently delayed and diminished in severe AS (parvus et tardus).

This finding is often absent in older patients with less compliant vasculature. In these circumstances, palpation of the brachial artery may reveal this finding.

25
Q

Describe some other diagnostic factors

A

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

May be accentuated by left bundle branch block.

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

bleeding
Some patients may develop an acquired von Willebrand deficiency that predisposes to bleeding and is caused by turbulent flow across the stenotic valve.[28] Patients may complain of epistaxis or bruising. They are also more likely to develop chronic gastrointestinal bleeding that is associated with angiodysplasia

26
Q

List some common risk factors

A

Age>60 years
Congenitally bicuspid aortic valve
Rheumatic Heart Disease
CKD

27
Q

List some weaker risk factors

A

radiotherapy
AS is a rare complication of mediastinal radiotherapy that presents at least 2 decades following treatment.[15]

high low-density lipoprotein (LDL) cholesterol
Retrospective studies have demonstrated that high LDL-cholesterol levels are associated with AS, and several small studies have found that statin therapy targeting LDL-cholesterol slows the haemodynamic progression of AS.[16] However, these findings were not verified in a large prospective study.[17]

hyperlipoproteinaemia
Familial hypercholesterolaemia is a rare genetic disorder that can contribute to early-onset calcification of the aortic valve and the aortic root.[18]

28
Q

Describe interpretation of the transthoracic echocardiogram

A

Transthoracic Doppler echocardiography is the best test for the initial diagnosis and subsequent evaluation of AS. The sensitivity and specificity of the test are high.[29]

The American College of Cardiology/American Heart Association (ACC/AHA) recommends transthoracic echocardiography when there is an unexplained systolic murmur, a single second heart sound, a history of a bicuspid aortic valve, or symptoms that might be due to AS.[20]

Result:
elevated aortic pressure gradient; measurement of valve area and left ventricular ejection function

29
Q

Describe interpretation of the ECG

A

The ECG is abnormal in >90% of patients with AS, with the most common abnormality being left ventricular hypertrophy (LVH) due to pressure overload.

Result:
may demonstrate left ventricular hypertrophy and absent Q waves, atrioventricular block, hemiblock, or bundle branch block

30
Q

Describe interpretation of the cardiac MRI

A

Cardiac MRI (cMRI) provides detailed, dynamic images of the heart. It allows for analysis of cardiac function and haemodynamics. Furthermore, cMRI is especially useful when trying to distinguish between true valvular stenosis and subvalvular stenosis related to a subvalvular membrane.

Due to the cost and complexity of cMRI, transthoracic echocardiography remains the preferred test for evaluating AS, but cMRI is a good option when echocardiography fails to yield quality images.

Result:
demonstrates stenotic aortic valve

31
Q

Describe interpretation of aortic catheterisation

A

Catheterisation allows for direct measurement of the pressure gradient. The sensitivity and specificity of the test are high.

Due to the invasive nature, this test is useful for diagnosis only when the echocardiogram is inconclusive or discrepant from other findings.

Result;
Elevated aortic pressure gradient

32
Q

Describe interpretation of ECG exercise stress testing

A

Consider in asymptomatic patients and those with equivocal symptoms.

Many experts advocate the use of symptom-limited exercise testing in asymptomatic patients with severe AS as a means of risk-stratification for the development of symptoms, and for the need for surgery.

Exercise echocardiography may add additional diagnostic and prognostic information.[23][24]

Symptomatic patients should not have exercise testing and should be referred for valve replacement.

Result:
asymptomatic patients: a positive exercise stress test is defined as the onset of symptoms, ST changes, an abnormal blood pressure response, or complex ventricular arrhythmia

33
Q

Describe interpretation of the dobutamine stress echo

A

Useful for the population of patients with a low transvalvular gradient and left ventricular systolic dysfunction (low ejection fraction), in order to identify pseudostenosis and the presence of contractile reserve.

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

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

Result:
may demonstrate pseudostenosis and presence of contractile reserve

34
Q

Describe the signs and symptoms of aortic sclerosis

A

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

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

35
Q

Describe the difference between aortic stenosis and aortic sclerosis

A

Transthoracic Doppler echo will demonstrate no significant pressure gradient (<5 mmHg) across the aortic valve with aortic sclerosis.

36
Q

Describe the signs and symptoms of ischaemic heart disease

A

AS and coronary artery disease frequently co-exist and it is difficult to determine their relative contributions to cardiac complaints when both are present in the same patient.

In practice, for patients with severe AS and concomitant coronary artery disease, both are treated at the time of surgery

37
Q

Describe how investigations will reveal differences between aortic stenosis and ischaemic heart disease

A

In ischaemic heart disease, common ECG findings include Q waves in contiguous leads, and the echocardiogram often shows segments of the myocardium with abnormal systolic thickening or wall motion abnormalities.

In AS, Q waves are absent in the ECG; regional wall motion abnormalities are absent on the echocardiogram; and coronary arteries are normal upon cardiac catheterisation.

38
Q

Describe the signs and symptoms of hypertrophic cardiomyopathy

A

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

Hand gripping and squatting manoeuvres are helpful in distinguishing HCM from AS. These manoeuvres do not greatly affect the intensity of the murmur of AS; however, hand gripping, which increases afterload, will soften the HCM murmur. Standing up after squatting will decrease cardiac return and ventricular preload, which increases the murmur of HCM.

39
Q

Describe how investigations will distinguish HCM from aortic stenosis

A

Typical echocardiography findings include asymmetrical hypertrophy with the septal wall most prominently affected. Dynamic obstruction is observed at the subvalvular level, and calcification of the valve is often absent, especially in younger patients.[31]

40
Q

Describe stage A of aortic stenosis

A

Stage A: at risk of AS

Valve anatomy: bicuspid aortic valve or other congenital valve anomaly, or aortic valve sclerosis
Maximum aortic velocity <2 m/s
No haemodynamic consequences
No symptoms

41
Q

Describe stage B of aortic stenosis

A

Stage B: Progressive AS

Mild AS: maximum aortic velocity 2.0 to 2.9 m/s or mean pressure gradient <20 mmHg
Moderate AS: maximum aortic velocity 3.0 to 3.9 m/s or mean pressure gradient 20-39 mmHg
Valve anatomy of mild-to-moderate leaflet calcification of a bicuspid or trileaflet valve with some reduction in systolic motion, or rheumatic valve with commissural fusion
Haemodynamic consequences: normal left ventricular ejection fraction (LVEF); early left ventricular (LV) diastolic dysfunction may be present
No symptoms

42
Q

Describe stage C of aortic stenosis

A

Maximum aortic velocity ≥4 m/s or mean pressure gradient ≥40 mmHg
Valve area typically ≤1.0 cm² (or indexed valve area ≤0.6 cm²/m²)
Very severe AS: maximum aortic velocity ≥5 m/s or mean pressure gradient ≥60 mmHg
Anatomy shows severe leaflet calcification or congenital stenosis with severely reduced leaflet opening
Haemodynamic consequences:
Stage C1: mild LV diastolic dysfunction, mild left ventricular hypertrophy (LVH), normal LVEF
Stage C2: LVEF <50%
No symptoms
Exercise testing is reasonable to confirm lack of symptoms in stage C1 AS

43
Q

Describe stage D1 of aortic stenosis

A

Stage D: Symptomatic severe AS

Stage D1: Symptomatic severe high-gradient AS
Maximum aortic velocity ≥4 m/s or mean pressure gradient ≥40 mmHg
Valve area typically ≤1.0 cm² (or indexed valve area ≤0.6 cm²/m²), but may be larger with mixed AS/aortic regurgitation (AR)
Anatomy: severe leaflet calcification or congenital stenosis with severely reduced leaflet opening
Haemodynamic consequences: LV diastolic dysfunction and LVH; pulmonary hypertension may be present
Symptoms of exertional dyspnoea or decreased exercise tolerance, exertional angina, exertional syncope or presyncope

44
Q

Describe stage D2 of aortic stenosis

A

Stage D2: Symptomatic severe low-flow/low-gradient AS with reduced LVEF
Valve area ≤1.0 cm² with resting maximum aortic velocity <4 m/s or mean pressure gradient <40 mmHg
Dobutamine stress echo shows valve area ≤1.0 cm² with maximum aortic velocity ≥4 m/s at any flow rate
Anatomy: severe leaflet calcification with severely reduced leaflet motion
Haemodynamic consequences: LV diastolic dysfunction, LVH, and LVEF <50%
Symptoms of heart failure, angina, syncope, or presyncope

45
Q

Describe stage D3 of aortic stenosis

A

Stage D3: Symptomatic severe low-gradient AS with normal LVEF or paradoxical low-flow severe AS
Valve area ≤1.0 cm² with maximum aortic velocity <4 m/s or mean pressure gradient <40 mmHg
Indexed valve area ≤0.6 cm²/m² and stroke volume index <35 mL/m² measured when patient is normotensive (systolic blood pressure <140 mmHg)
Anatomy: severe leaflet calcification with severely reduced leaflet motion
Haemodynamic consequences: increased LV wall thickness, small LV chamber with low stroke volume, restrictive diastolic filling, and LVEF ≥50%
Symptoms of heart failure, angina, syncope, or presyncope

46
Q

Summarise the pathophysiology of aortic stenosis

A

Aortic stenosis usually occurs as a result of calcification of a normal aortic valve or congenitally bicuspid aortic valve (tends to occur at a younger age). It can also result from rheumatic heart disease. The stenosed valve increases the afterload on the left ventricle, increasing the force required to eject blood into the aorta. This causes hypertrophy of the left ventricle. The reduction in cardiac output can result in breathlessness and inadequate perfusion of tissues such as the brain (causing syncope). Reduced myocardial perfusion combined with the increased myocardial oxygen demand can also cause angina.