QuestionSBA
QuestionChoice
HOCM is classically a disease of the young. It does cause an ejection systolic murmur similar in quality to aortic stenosis, but it would be rare for a patient with significant HOCM to survive to 79 years of age without diagnosis and intervention
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c
Hypertrophic obstructive cardiomyopathy (HOCM)
QuestionChoice
Aortic regurgitation is an early diastolic murmur that may become pan systolic as the regurgitation becomes more severe. It is heard loudest at the left sternal edge (representing the direction of regurgitant blood flow from the faulty valve)
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b
Aortic regurgitation
QuestionChoice
Pulmonary stenosis is a much rarer cause of an ejection systolic murmur, particularly in adults (most commonly caused by congenital heart disease and identified early on). It is also heard best in the pulmonary, rather than aortic region
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d
Pulmonary stenosis
QuestionChoice
Aortic stenosis is the cause of most valvular heart disease in the developed world and most commonly presents in those above 70 years of age. It occurs with age as the aortic valve becomes calcified, and is related to the usual cardiovascular risk factors of smoking, hypercholesterolaemia, obesity, diabetes and hypertension. In addition, congenital bicuspid valves are more prone to becoming stenosed at an earlier age - these patients may present in their 50's, or sometimes even younger
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a
Aortic stenosis
QuestionChoice
Mitral regurgitation is a pan systolic murmur which is sometimes described as "blowing" in character. It is also heard loudest at the apex
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e
Mitral regurgitation
Concept
Chapter
# Summary

Aortic stenosis (AS) is characterised by the narrowing of the aortic valve, which restricts blood flow from the left ventricle to the aorta and the rest of the body. It is a common valvular pathology, with severe aortic stenosis having an estimated prevalence of 3% in those over 75 years old. The condition can be caused by senile calcification, congenital bicuspid valve, rheumatic heart disease, or supravalvular stenosis. Mild-to-moderate aortic stenosis may be asymptomatic, but severe cases present with symptoms such as syncope, angina, and dyspnoea. Diagnosis is confirmed through echocardiography. Management of asymptomatic AS is usually conservative and patients require regular echocardiograms. Symptomatic AS requires medical treatment to optimise symptoms and consideration for surgical or interventional interventions. Severe, symptomatic aortic stenosis carries a high risk of mortality without intervention, and prompt treatment is crucial. 

# Definition 

Aortic stenosis (AS) refers to the narrowing and tightening of the aortic valve leading to reduced blood flow from the left ventricle into the aorta and ultimately to the rest of the body. 

# Epidemiology 

For individuals over 75 years old, the prevalence of severe aortic stenosis is estimated at 3%. It is a common valvular pathology. 

# Pathophysiology

Normally, the aortic valve opens to allow blood to be pumped from the left ventricle into the aorta and to the rest of the body during systole. In aortic stenosis, there is a narrowing of the aortic valve which means that the left ventricle has to generate more pressure to enable sufficient blood to cross the aortic valve and pass into the aorta. Initially, this leads to left ventricular hypertrophy as the left side of the heart compensates for the narrowing. Over time, the left ventricle can no longer compensate and the left ventricle will start to enlarge, the ejection fraction will reduce, and this will ultimately leads to reduced cardiac output.

# Cause 

Causes of AS include: 

* Senile calcification: most common cause in those >65y/o. 
* Congenital bicuspid valve: most common cause in those <65y/o.
* Rheumatic heart disease 
* William's syndrome: supravalvular stenosis 

# Symptoms

Like with all valvular pathologies, mild-to-moderate aortic stenosis may be asymptomatic and may be picked up incidentally on cardiac auscultation or on echocardiogram. 

The symptoms of severe AS can be remembered by the mnemonic 'SAD'. 

* **S**yncope 
* **A**ngina 
* **D**yspnoea 

Other symptoms include: pre-syncope, palpitations, left ventricular heart failure symptoms (exertional dyspnoea, orthopnoea, PND) or can present in cardiac arrest/sudden cardiac death. 

# Signs

* Slow-rising carotid pulse
* Narrow pulse pressure
* Heaving, non-displaced apex beat (can be displaced if there is left ventricular hypertrophy)
* Ejection systolic murmur
  * Heard best at the second intercostal space on the right
  * Can be described as "harsh"
  * Radiates to the carotids
* Soft S2 heart sound: absent S2 corresponds with severity
* Ejection click may be heard in some cases (early systolic)

# Investigations

## Bedside

* **ECG:** LVH, left axis deviation, and poor R wave progreesion. 

## Imaging

* **CXR:** cardiomegaly and evidence of pulmonary oedema. Occasionally, a calcified aortic valve is visible. 
* **Echocardiogram: definitive diagnosis**. 
	* Definitive diagnosis of aortic stenosis. 
	* Severity of AS can be quantified with doppler echocardiography. AS is classified as severe if it meets the following parameters: 
		* Peak gradient > 40 mmHg (note, in severe left ventricular dysfunction, a low peak gradient can be falsely reassuring)
		* Valve area < 1.0cm x<sup>2</sup>
		* Aortic jet velocity >4 m/s
* **Exercise testing:** may be used in physically active patients to assess the true severity of asymptomatic patients with echocardiography confirmed AS. 
* **Cardiac MRI:** can be used to provide additional, more details information regarding valve morphology, dimensions of the aortic root and the extent of valve calcification. 

# Management

## Conservative 

Timing of intervention is crucial as many cases remain asymptomatic and stable and may not require treatment at all. 

Patients who do not meet the criteria for intervention should have regular echocardiography follow-up, with severe AS being monitored every 6 months, mild-to-moderate AS monitored yearly, and younger patients can be monitored every two to three years.

## Medical 

Medical management of AS involves symptom management of left ventricular failure with diuretics and optimising heart failure medications with beta-blockers and ACE-I etc. Isolated medical therapy should only be used in those who are not suitable for intervention. 

## Surgical and Interventional 

Intervention in AS is indicated in the following patients:

* All patients with symptomatic aortic stenosis 
* Asymptomatic patients with a left ventricular ejection fraction (LVEF) < 55%
* Asymptomatic patients with an LVEF > 50% who are physically active, and who have symptoms or a fall in blood pressure during exercise testing
* Asymptomatic patients with an LVEF > 50% who have the following risk factors
  * Aortic valve peak velocity > 5m/s x<sup>2</sup>
  * Severe calcification and peak velocity progression >= 0.3m/s x<sup>2</sup>
  * Markedly elevated BNP levels (more than twice upper limit) without other explanation
  * Severe pulmonary hypertension (pulmonary artery systolic pressure > 60mmHg)
  * Aortic valve area less than 0.6 cm<sup>2 


Choices of intervention are **transcatheter aortic valve implantation (TAVI)** or **surgical aortic valve replacement (SAVR)**.

* TAVI is favoured with patients with severe comorbidities, previous heart surgery, frailty, restricted mobility, and those older than 80 years of age.
* SAVR is favoured for patients who are low risk and younger.

# Complications

Aortic stenosis if left untreated can lead to LV failure. It is also implicated in sudden cardiac death. 

# Prognosis 

Severe, symptomatic AS patients are at a very high risk of sudden-death. It is estimated that these patients have a mortality rate of 50% at 1 year. It is essential that these patients are identified and treated swiftly. 

# Aortic sclerosis

Aortic sclerosis is an asymptomatic condition that can be incidentally revealed through physical examination or via echocardiogram. It is caused by age-related senile degeneration of the valve.

## Clinical findings

Classic findings are an ejection systolic murmur that _does not radiate to the carotids_, there is normal S2, pulse character and volume.

It can be defined as an irregular aortic leaflet thickening and focal increased echogenicity, but is distinguished from aortic stenosis because there is no impairment of valve leaflet excursion, and peak doppler velocities are normal or only minimally elevated (markedly elevated in stenosis)

# NICE Guidelines

[NICE Guidelines for Valvular Heart Disease](https://www.nice.org.uk/guidance/ng208/chapter/recommendations##References:)

# References 

[BMJ Aortic Stenosis Summary](<https://www.bmj.com/content/380/bmj-2022-070511>)
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A 79 year old man with no prior history of heart disease is examined by his GP. They note an ejection systolic murmur, heard loudest in the aortic region.

What is the most likely cause of this murmur?
a