Valvular diseases and heart sounds

Question 1

What are the causes of aortic stenosis?

Answer 1

Calcification of tri-leaflet aortic valve
Bicuspid aortic valve stenosis
Congenital abnormalities (supravalvular aortic stenosis, Unicuspid valve)
Degeneration (why its most common in old age)
Rheumatic aortic stenosis

Question 2

Which other conditions is a Bicuspid aortic valve (BAV) also associated with?

Answer 2

aortic coarctation, Aortic root dilatation and, potentially, aortic dissection

Question 3

What are the risk factors for calcified aortic stenosis?

Answer 3

hypercholesterolaemia
hypertension
Smoking
diabetes

Question 4

What happens to the heart over time in those with aortic stenosis?

Answer 4

The heart can not pump out a normal amount of end diastolic blood

So it heart undergoes hypertrophy to compensate

The hypertrophy may eventually lead to diastolic dysfunction through impaired relaxation and reduced compliance

Causes diastolic heart failure with features of systolic heart failure

Heart failure can follow with signs of left sided heart failure

Or angina can develop

Question 5

What are the clinical signs and symptoms associated with aortic valve stenosis?

Answer 5

Syncope (exertional)
Angina (chest pain)
Dyspnoea
Signs and symptoms of of congestive heart failure

Ejection systolic murmur, radiating to the carotids

Sustained apex (only displaced if their is left sided heart failure)

Slow rising pulse
narrow pulse pressure

4th heart sound (caused by the atria contracting against stiff, hypertrophied ventricles

May be an ejection click

Quite second heart sound (sever aortic stenosis)

Splitting of second heart sound- aortic valve will open later and close after the pulmonary valve
(aortic valve opens- pulmonary valve opens and closes- aortic valve closes)

Question 6

Which bleeding disorder can be caused by aortic stenosis? Why does this disorder develop?

Answer 6

Von Willebrand syndrome

Due to turbulent flow along the aortic valve. This causes a high sheering force which causes structural changes in the shape of the Von Willebrand protein located in the blood cell.

This causes clotting abnormalities

Question 7

Which investigation is used to diagnose aortic stenosis other valvular disease?

Answer 7

Echocardiogram

Question 8

Which other investigations would you conduct in someone suspected of having aortic stenosis?

Answer 8

BP
ECG
FBC
U and E's 
Cholesterol
Clotting screen 

Chest X ray
Echo
Cardiac catheter ( can assess: valve gradient; LV function; coronary artery disease; risks: emboli generation)

Question 9

On an ECG what might you see in someone with aortic stenosis?

Answer 9

Findings indicating left ventricular hypertrophy (Deep S waves in V1 and V2, Tall R waves in V5 and V6)

A negative P wave in lead V1 - caused by atrial enlargement seen in sever cases of aortic stenosis

Question 10

How is aortic stenosis treated?

Answer 10

Stenosis can be treated with a valvotomy - Stenotic valve leaflets are forced apart (percutaneous balloon valvotomy or open valvotomy)

Symptomatic patients or those with sever AS and deteriorated ECG - Valve replacement

If not fit for surgery
percutaneous valvuloplasty/replacement (TAVI = transcatheter aortic valve implantation) may be attempted. An expandable valve is used

Question 11

What is the pulse pressure?

Answer 11

Difference between the systolic and diastolic pressures

Question 12

List 3 causes of Ejection systolic murmur

Answer 12

Aortic stenosis or sclerosis
Pulmonary stenosis
HOCM

Question 13

List 3 causes of Ejection systolic murmur

Answer 13

Aortic stenosis or sclerosis
Pulmonary stenosis
HOCM (Hypertrophic obstructive cardiomyopathy)

Question 14

When an doppler echo cardiogram is done for aortic stenosis, what is Echo assessing?

Answer 14

The gradient across the valve

The valve area

Question 15

When a doppler echo cardiogram is done for aortic stenosis, what is the Echo assessing?

Answer 15

The pressure gradient across the valve

The valve area

Left ventricular hypertrophy or dilation
Left ventricular contractility in systole and any stiffness or non compliance is diastole

Question 16

What are the 2 types of aortic valve replacements a patient can have? Which types of patients would be best suited for each valve type? How are patients with these valves treated post surgery?

Answer 16

Mechanical valve - Patient requires long term anticoagulation. Has a long life span reducing the need for a second operation. Best for young patients

Bioprosthetic valve- no need for long-term anticoagulation. Limited life span (10 years). A repeat operation is more likely. Best suited for older patients

Question 17

What is aortic regurgitation?

Answer 17

Incompetent aortic valve causing a regurgitant flow of blood in diastole.

Question 18

At what age is aortic regurgitation most common?

Answer 18

fourth and sixth decades of life

Affects males 3x more than women

Question 19

What are the causes of aortic regurgitation?

Answer 19

Valve leaflets -

Bicuspid valve/other congenital abnormality
Degeneration
Infective Endocarditis
Rheumatic heart disease (common in developing world)

Aortic root- 
Aortitis (inflammation of aortic route)
Aortic root dissection (Stanford A)
Dilated aortic root
Connective tissue diseases - includes rheumatoid arthritis

Question 20

In Endocarditis, which infective causes can lead to aortic dissection?

Answer 20

Strep. viridans, Staph. aureus, Enterococci

Question 21

Which connective tissue disorders are associated with aortic regurgitation? What is the defective in these conditions and how should people with these conditions be monitored with regards to their risk of developing the condition?

Answer 21

Marfan’s syndrome - caused by a defect in the FBN1 gene.

Ehlers-Danlos syndrome - caused by collagen defects.

Both conditions can cause an aortic root dilation which can lead to aortic regurgitation.

Aortic root diameter should be monitored in these patients

Question 22

Which conditions can cause aortitis?

Answer 22

Chronic inflammatory conditions e.g.

Rheumatoid arthritis (RA) and ankylosing spondylitis (AS),

Question 23

Describe acute aortic regurgitation?

Answer 23

Acute -

• Medical emergency
• An acute rise in left atrial pressure results in pulmonary oedema & cardiogenic shock
• Regurgitation of blood during diastole causes an increase in the left ventricular end-diastolic volume (and pressure)

Effects -
Reduced coronary flow - coronaries fill predominantly during diastole, regurgitant flow at this time reduces filling. Results in angina or in severe cases myocardial ischaemia.

Increased end-diastolic pressure - causes increased pulmonary pressures with resulting pulmonary oedema and dyspnoea. In severe cases, cardiogenic shock may occur.

Question 24

What are the causes of acute aortic regurgitation?

Answer 24

Infective endocarditis
Rheumatic fever
Aortic dissection

Question 25

What is chronic aortic regurgitation?

Answer 25

- Compensatory changes have taken place - Patients may remain asymptomatic for many decades - Valvular incompetence develops slowly - Regurgitation of blood during diastole causes an increase in the left ventricular end-diastolic volume - This leads to systolic and diastolic dysfunction, left ventricular dilatation develops with eccentric hypertrophy. - The dilation allows for an increased stroke volume compensating for regurgitant flow supported by the ventricular hypertrophy. These changes maintain ejection fraction, with a greater preload leading to greater contractility - Eventually further increases in preload cannot be met by greater contractility and heart failure develops.

Question 26

What is the cause of chronic aortic regurgitation?

Answer 26

Rheumatic heart disease Arthritides Bicuspid valve Connective tissue disorders

Question 27

What are the signs and symptoms of acute and chronic aortic regurgitation?

Answer 27

Acute - - Sudden Dyspnea - Chest pain (may be MI, angina or aortic dissection) - Bi basal crackles - Raised JVP Essential acute features of hear failure Chronic- - Palpitations - Angina - Dyspnoea - collapsing -water hammer pulse - wide pulse pressure - Displaced apex beat - Ejection diastolic mummer - Soft S1 and S2

Question 28

What are the Eponymous signs for aortic regurgitation?

Answer 28

de Musset’s - head nodding with the heart beat. Quincke’s - pulsation of nail beds. Traube’s - pistol shot femorals. Duroziez’s - to and fro murmur heard when stethoscope compresses femoral vessels. 2cm proximal to stethoscope = systolic murmur. 2cm distal to stethoscope = diastolic murmur Müller’s - pulsation of uvula.

Question 29

Which best side/ blood tests would you do for aortic regurgitation?

Answer 29

Bedside- Observations Blood pressure ECG: left ventricular hypertrophy (deep S-waves in V1 and V2, tall R-waves in V5 and V6) in chronic AR ``` Bloods- FBC U&Es Cholesterol Clotting ```

Question 30

Which imaging test would you do for someone with aortic regurgitation and what would you find in these tests?

Answer 30

Echocardiogram (transthoracic or transoesophageal)- - left ventricular hypertrophy (chronic Aortic regurgitation) - Origin and width of regurgitation jet - Detection of aortic valve pathology Chest x ray - - Cardiomegaly - Sighns of heart failure - May show dilated ascending aorta CT/MRI- - MRI may be used to estimate the regurgitant fraction - In patients with aortic dilatation, gated multi-slice CT is the imaging of choice to characterise aortic dilatation and the maximum diameter. Angiography - - In patients with chronic AR undergoing surgery, pre-operative angiography is indicated. Typically this is in the form of coronary angiography (invasive procedure) to assess for concomitant coronary artery disease that may require bypass.

Question 31

How is acute aortic regurgitation treated?

Answer 31

Aortic valve replacement or repair should be performed as soon as possible.

Question 32

How is chronic aortic regurgitation treated?

Answer 32

Reduce systolic hypertension e.g Ace inhibitors Echo every 6-12 months to monitor Surgical management required if - - Sever AR with significant enlargement of the ascending aorta or - Symptomatic severe AR or - Severe AR with LVEF ≤ 50% or LVEDD > 70mm or LVESD > 50mm (may be adjusted for body size) - Marfan's with aortic root disease with a maximal ascending aorta diameter ≥ 50mm - infective endocarditis refractory to medical therapy Surgical option = mechanical or bio prosthetic valve replacement ascending aorta dilatation management depends on the anatomical pattern, patients may need root replacement with reimplantation of the coronary vessels either with preservation of the native valve or with replacement or a supracommissural tube graft replacement.

Question 33

What is mitral valve stenosis?

Answer 33

Valvular obstruction to flow from the left atrium to left ventricle due to stenosis of the mitral valve

Question 34

What are the causes of mitral stenosis?

Answer 34

``` Rheumatic fever (Most common) Congenital MS Mitral annular calcification Radiation associated MS Carcinoid associated valve disease Fabry's disease mucopolysaccharidoses, endocardial fibroelastosis Prosthetic valve ```

Question 35

What is the normal mitral valve orifice area? At which point do symptoms begin to develop?

Answer 35

Normal- ~4–6cm | Symptoms present >2cm

Question 36

What is the pathophysiology of mitral stenosis?

Answer 36

There is raised pressure in the atria that may occur only during exercise or in more severe disease at rest. Left atrial enlargement occurs in the presence of chronically elevated atrial pressures predisposing patients to both atrial fibrillation and atrial thrombosis. Raised atrial pressures translate to raised pulmonary venous pressures and pulmonary hypertension which can eventually lead to right sided heart failure.

Question 37

What are the signs and symptoms of mitral stenosis?

Answer 37

Exertional dyspnoea Haemoptysis - as a result of vascular congestion and can result from the rupture of thin walled bronchial veins. Chest pain is occasionally seen, often a result of angina from underlying coronary artery disease and hypertrophic myocardium having increased oxygen demands. A mid-diastolic murmur is characteristic - best heard with the bell of the stethoscope with the patient lying on their left side whilst breath is in held expiration. Ortner syndrome - a horse voice that occurs secondary to left atrial enlargement causing a left recurrent laryngeal nerve palsy. ``` Fatigue Palpitations Atrial fibrillation Mitral facies (malar flush) Pulmonary hypertension: Right ventricular heave Prominent a-wave Right heart failure: Raised JVP Peripheral oedema Hepatomegaly ``` Dysphagia Bronchial obstruction Systemic emboli Malar flush on cheeks (decreased cardiac output) Low volume pulse Tapping, non displaced, apex beat Right ventricular heave (parasternal heave) can also be caused by atrial enlargement but very rarely Loud S1 sound (opening snap) Severity: the more severe the stenosis, the longer the diastolic murmur, and the closer the opening snap is to S2.

Question 38

Patients with mitral stenosis are at greater risk of Thromboembolism. Why?

Answer 38

Clots that develop in a dilated left atrium, often in the presence of atrial fibrillation, may throw off emboli into the systemic circulation. As such patients may present with a stroke

Question 39

Which tests are conducted to assess mitral stenosis and what would they show?

Answer 39

Echocardiogram is diagnostic and used to assess disease severity. Valve area, mean gradient, PA pressure Transoesophageal echocardiogram- an be performed to look for left atrial thrombosis either after an embolic episode (e.g. stroke) or prior to percutaneous mitral commissurotomy. Chest X ray - Left atrial enlargement Signs of pulmonary hypertension and right sided heart failure ECG- Atrial fibrillation (not always) Signs of left atrial enlargement - p mitrale, a broad, notched P wave, negative component in V1 Right sided hypertrophy present = right axis deviation, tall R waves in V1 Stress testing - Stress testing is sometimes used in those who are asymptomatic or have minor symptoms as well as those whose symptoms are discordant with echo findings. This may take the form of an exercise stress echo or dobutamine stress test.

Question 40

Which findings on an x ray would indicate atrial enlargement?

Answer 40

Double right heart border Splayed trachea Prominence of the atrial appendage

Question 41

How is mitral stenosis treated?

Answer 41

Percutaneous mitral commissurotomy - a balloon delivered by a catheter enters at the right femoral vein. This balloon is then inflated in various stages to help alleviate the stenosis. In those not suitable for PMC, open surgery may be considered and discussed with the patient. Patient with AF or paroxysmal AF can tend to follow the routine management of this condition. In stable patients pre-intervention, cardioversion is not indicated as the results are not sustained. Patients should receive appropriate anticoagulation. patients with ‘moderate to severe mitral stenosis and persistent atrial fibrillation should be kept on vitamin K antagonist (VKA) treatment and not receive NOACs. Patients in sinus rhythm may obtain anticoagulation if thrombus is in the left atrium and history of systemic embolism or if Left atrium is enlarged

Question 42

What are the contraindications for percutaneous mitral commissurotomy?

Answer 42

Valve area >1.5cm2 Left atrial thrombus Absence of commissural fusion Severe or bi-commissural calcification More than mild mitral regurgitation Severe concomitant aortic stenosis requiring surgery Severe combined tricuspid regurgitation/stenosis requiring surgery Concomitant coronary artery disease requiring surgery

Question 43

Under which circumstances is percutaneous mitral commissurotomy indicated?

Answer 43

PMC tends to be reserved for patients with clinically significant MS and a valve area < 1.5cm2 in those who are symptomatic or at high-risk of embolism or haemodynamic decompensation.

Question 44

What is mitral regurgitation?

Answer 44

Incompetence of the mitral valve causing blood to leak through due to abnormalities to the valve leaflets, subvalvular apparatus or left ventricle.

Question 45

What is primary and secondary mitral valve regurgitation?

Answer 45

Primary- pathology affecting components of the valve itself. Degenerative disease is the most common cause. Secondary- caused by changes to left ventricular geometry. This results in distortion of the subvalvular apparatus and valve leaflets. Dilated and ischaemic cardiomyopathies are the most common cause.

Question 46

What is chronic mitral regurgitation?

Answer 46

There is gradual worsening of the regurgitant fraction that initially allows for compensatory mechanisms to occur. Eventually failure may result and the patient enters a decompensated state: - Compensate - Left ventricle and left atrium dilate. - The compliant and dilated left ventricle undergoes eccentric hypertrophy and is able to maintain a larger stroke volume and as such ejection fraction. - The compliant and dilated left atrium prevents rises in atrial and therefore pulmonary pressures. Decompensated - - eventually such changes cannot maintain normal cardiac function and the remodelling becomes increasingly pathological - The heart fails, ejection fraction falls and pulmonary pressures rise

Question 47

What is acute mitral regurgitation?

Answer 47

There are fast and significant changes to flow without time for any adaptation or remodelling to occur as is seen in chronic MR. The new regurgitation causes increased pressure within a non-compliant left atrium. This causes a rise in pulmonary pressure resulting in pulmonary oedema Ejection fracture falls as blood goes back through the regurgitating valve in stead of going through the aortic valve. A tachycardic response may be raised to help compensate for the reduced ejection fraction in an attempt to return the cardiac output to normal. This is rarely sufficient and cardiogenic shock can occur.

Question 48

What are the clinical features of acute mitral regurgitation?

Answer 48

- Rapid development of heart failure in inadequate cardiac output - Flash pulmonary oedema - Patient may be in shock and breathless - Condition is potentially life threatening

Question 49

What are the clinical features/ symptoms of chronic mitral regurgitation?

Answer 49

- May be asymptomatic for many years - Heart failure will cause symptoms to develop - symptoms tend to involve dyspnoea and orthopnoea that results from pulmonary hypertension - Fatigue and malaise are common. - Peripheral oedema due to right sided heart failure

Question 50

What are the signs of mitral regurgitation?

Answer 50

- Laterally displaced apex beat - Systolic thrill in sever cases - Pan systolic murmur - Sighs of heart failure (e. g bi basal crackles, peripheral oedema)

Question 51

Which investigations would you conduct for someone with mitral regurgitation and what would you see in each?

Answer 51

Chest X - ray- - left atrial (double right heart dorder) and ventricular enlargement - Cardiomegaly (not see in acute cases) - Signs of pulmonary oedema ECG- - In acute case - normal or show indications of recent myocardial infarction - Chronic condition p-mitrale - a broad, notched p-wave with a negative component in V1 - (reflecting left atrial enlargement) and signs of left ventricular hypertrophy. - Arrhythmia's, most commonly atrial fibrillation, are sometimes present. Echocardiogram - visualisation of the incompetent valve and can confirm the underlying aetiology. Left atrial and ventricular enlargement may be seen in chronic MR. Cardiopulmonary Exercise testing - assess a patients overall functional capacity. Exercise echocardiography is used to demonstrate changes in MR during exercise. Cardiac MRI- - may be used when echocardiogram is inadequate. Cardiac catheterisation - used for evaluation of the coronary vessels prior to valvular surgery. Right sided catheterisation can be used to confirm pulmonary hypertension.

Question 52

How is primary mitral regurgitation managed?

Answer 52

Acute - Medical stabilisation- - Sodium nitroprusside - reduces afterload - Inotropic agents e.g Dobutamine and an intra-aortic balloon - hypotension Surgery (depends on patients clinical state and underlying aetiology) - - Surgical repair - infective endocarditis and chordal rupture - Surgical replacement - papillary muscle rupture Chronic - Medical therapy - - In patients with chronic MR and heart failure, ACE inhibitors, beta-blockers and spironolactone may all be considered - Cardiac resynchronisation therapy (CRT) is used when appropriate - If AF control rate with beta blockers and anticoagulated. History of embolism = prosthetic valve Surgical - considered in symptomatic patients with a LVEF > 30%. aim to repair or replace the valve before LV is irreversibly impaired. - Can also be considered in other patients - specialist call - Other surgical measures include ventricular assist devices, cardiac restraint devices and heart transplantation.

Question 53

How is secondary mitral regurgitation managed?

Answer 53

- management should be guided by MDT | - Medical therapy should follow heart failure management. CRT should be considered when appropriate

Question 54

What is the most common valvular pathology?

Answer 54

Mitral valve prolapse

Question 55

What are the signs and symptoms of Mitral valve prolapse?

Answer 55

- Usually asymptomatic. May develop atypical chest pain, palpitations, and autonomic dysfunction symptoms Signs Mid-systolic click and/ or a late systolic murmur

Question 56

What are the complications of Mitral valve prolapse?

Answer 56

Mitral regurgitation, cerebral emboli, arrhythmias, sudden death

Question 57

How is Mitral valve prolapse diagnosed and treated?

Answer 57

Echo is diagnostic. ECG may show inferior T-wave inversion. - blockers may help palpitations and chest pain. Surgery if severe MR.

Question 58

What are the causes of pulmonary stenosis?

Answer 58

- Usually congenital (Turner syndrome, Noonan syn- drome, Williams syndrome, Fallot’s tetralogy, rubella) - Acquired causes: rheumatic fever, carcinoid syndrome

Question 59

What are the signs and symptoms of pulmonary stenosis?

Answer 59

Dyspnoea; fatigue; oedema; ascites ``` Signs - Dysmorphic facies (congenital causes); - prominent a wave in JVP; - RV heave In mild stenosis, - ejection click - ejection systolic murmur (which radiates to the left shoulder); - widely split S2. ``` In severe stenosis, - murmur becomes longer and obscures A2. - P2 becomes softer and may be inaudible

Question 60

How is pulmonary stenosis Diagnosed?

Answer 60

ECG: Right axis deviation , P-pulmonale, RV Hypertrophy , RBBB ECHO CXR: prominent pulmonary arteries caused by post-stenotic dilatation Cardiac catheterization is diagnostic

Question 61

How is pulmonary stenosis treated?

Answer 61

Pulmonary valvuloplasty or valvotomy

Question 62

What is the causes and symptoms of pulmonary regurgitation?

Answer 62

Causes: Any cause of pulmonary hypertension Signs: Decrescendo murmur in early diastole at the left sternal edge

Question 63

What are the causes of Tricuspid regurgitation?

Answer 63

- Functional (RV dilatation; eg due to pulmonary hypertension induced by LV failure or PE); - rheumatic fever; - infective endocarditis (IV drug abuser); - carcinoid syndrome; - congenital (eg ASD, AV canal, Ebstein’s anomaly (downward displacement of the tricuspid valve - drugs (eg ergot- derived dopamine agonists, ; fenfluramine)

Question 64

What are the symptoms of Tricuspid regurgitation?

Answer 64

``` Symptoms - Fatigue; - hepatic pain on exertion (due to hepatic congestion); - ascites; - oedema - symptoms of the causative condition ``` Signs - Giant v waves - prominent y descent in JVP - RV heave; - pansystolic murmur, heard best at lower sternal edge in inspiration; - pulsatile hepatomegaly; - jaundice; - ascites

Question 65

How is Tricuspid regurgitation managed?

Answer 65

- Drugs: diuretics for systemic congestion; - Drugs to treat underlying cause. - Valve repair or replacement

Question 66

What is the cause of Tricuspid stenosis?

Answer 66

Causes: Main cause is rheumatic fever | congenital, infective endocarditis

Question 67

What are the symptoms and signs of Tricuspid stenosis?

Answer 67

Symptoms: Fatigue, ascites, oedema. Signs: Giant a wave and slow y descent in JVP opening snap, early diastolic murmur heard at the left sternal edge in inspiration. AF can also occur

Question 68

How is Tricuspid stenosis diagnosed and treated?

Answer 68

Diagnosis: Echo. Treatment: Diuretics; surgical repair.

Question 69

What are the ECG findings for pulmonary hypertension?

Answer 69

- Right axis deviation - Positive QRS complexes (‘dominant R waves’) in V1 and V2 suggesting right ventricular hypertrophy - ST depression and T-wave inversion in the right precordial leads (V1–3) suggesting right ventricular strain - Peaked P waves (P pulmonale) suggesting right atrial hypertrophy.

Question 70

When a patient has a dual pace maker, how will it show on an ECG?

Answer 70

Pacing spikes occur before each P wave and each QRS complex. Paced QRS complexes are broad as the impulse starts in the ventricles

Question 71

What causes a raised JVP with normal waveform?

Answer 71

Fluid overload, right heart failure

Question 72

What causes a fixed raised JVP with absent pulsation?

Answer 72

superior vena cava obstruction

Question 73

What causes a large a wave?

Answer 73

Pulmonary hypertension, pulmonary stenosis

Question 74

What causes a cannon a wave?

Answer 74

When the right atrium contracts against a closed tricuspid valve —complete heart block, single chamber ven- tricular pacing, ventricular arrhythmias/ectopics.

Question 75

What causes an absent a wave?

Answer 75

Atrial fibrillation

Question 76

What causes Large v waves?

Answer 76

Tricuspid regurgitation—look for earlobe movement

Question 77

What causes an absent JVP?

Answer 77

``` If there is reduced circulatory volume (eg dehydration, haemorrhage) the JVP may be absent. ```