Valvular Heart Disease

Now here is a topic that is close to every medical student’s heart 😉

I don’t know why, but us medical students seem to be obsessed with heart murmurs. There is nothing more satisfying, nothing makes us feel more like we are on our way to becoming doctors, than hearing a murmur with our stethoscopes. Or maybe it’s just me? Although, usually I just hear a whoosh but can’t really tell what kind of murmur it is, so I go for Ejection Systolic, because it is the most common. Whoosh. I have 95% confidence that this is an Ejection Systolic Murmur; now hand me my degree.


Valvular Heart Disease

Image result for heart valves
Definition:
abnormalities of the four heart valves- aortic, mitral, pulmonary and tricuspid valves. This can be a narrowing of the valve (stenosis) or insufficiency of the valve causing retrograde flow through the valve (regurgitation) when it is closed. There may also be prolapse, particularly mitral prolapse, where there is prolapse of the mitral valve leaflets into the left atrium during systole.

Note:

-Left sided valve disease is far more common than right sided

-The right sided heart murmurs more or less mirror the corresponding ones on the left.

Left sided murmurs are heard louder on expiration (when air LEaves the lungs), whilst right sided murmurs are heard louder on inspiration

-Systolic murmurs are louder than diastolic murmurs.

Aetiology/ risk factors:

Left Heart Valve Disease 

Systolic murmurs:

Aortic stenosis: senile calcification commonest (elderly), congenital (Bicuspid valve calcification, William’s syndrome), rheumatic heart disease

Mitral regurgitation: rheumatic heart disease, functional (LV dilation), annular calcification (elderly), infective endocarditis, mitral valve prolapse, ruptured chordae tendidae (2ry to IHD or cardiomyopathy),  papillary muscle dysfunction/rupture, connective tissue disorders (Marfan’s, Ehlers-Danlos, osteogenesis imperfecta), cardiomyopathy, congenital, appetite suppressants.

Mitral prolapse: occurs alone or with atrial-septal defects, patent ductus arteriosus, cardiomyopathy, Turner’s syndrome, Marfan’s syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, Wolff-Parkinson-White.

Diastolic murmurs:

Aortic regurgitation: Acute– infective endocarditis, ascending aortic dissection, chest trauma. Chronic– congenital (bicuspid valve), connective tissue disorders (Marfan’s, Ehlers- Danlos), rheumatic fever, Takayasu arteritis, rheumatoid arthritis, SLE, seronegative arthropathies, systemic hypertension, osteogenesis imperfecta, syphilitic aortitis, pseudoxanthoma elasticum, appetite suppressant.

AR is caused by aortic valve leaflet abnormalites or damage, as in infective endocarditis, or by aortic root/ ascending aorta dilation, as in systemic hypertension and aortic dissection.

Mitral stenosis: rheumatic heart disease, congenital, mucopolysaccharidoses, endocardial fibroelastosis, malignant carcinoid, prosthetic valve, SLE, rheumatoid arthritis, infective endocarditis, atrial myxoma.

 

Right Heart Valve Disease 

Systolic murmurs:

Pulmonary stenosis: usually congenital (Turner’s syndrome, Noonan’s syndrome, William’s syndrome, Fallot’s tetralogy, rubella). May be acquired in rheumatic fever and carcinoid syndrome.

Tricuspid regurgitation: usually functional and secondary to dilation of right ventricle (e.g. due to pulmonary hypertension). Other causes: valve prolapse, rheumatic heart disease, infective endocarditis (in IV drug abusers), carcinoid syndrome, congenital (Ebstein anomaly- malpositioned tricuspid valve; cleft valve in ostium primum defect), drugs (e.g. Ergot derived dopamine agonists), trauma, cirrhosis (long-standing).

Diastolic murmurs:

Pulmonary regurgitation: caused by any cause of pulmonary hypertension e.g. COPD, leading to dilation of the valve ring. Occasionally, infective endocarditis (usually in IV drug users).

Tricuspid stenosis: main cause is rheumatic fever, which almost always occurs with mitral or aortic valve disease, which tends to dominate the clinical picture Also: congenital, infective endocarditis.

Epidemiology:

-more than 50% of those who suffer acute rheumatic fever with carditis will later (10-20 years) develop chronic rheumatic valvular disease, predominantly affecting the aortic and mitral valves.

-acquired left sided valvular lesions are the most common.

Mitral prolapse is the most common valvular abnormality, prevalence ~5%. More common in young females.

Aortic stenosis affects ~3% of 75-year olds.

Mitral regurgitation affects ~5% of adults.

-tricuspid and pulmonary valve disease is uncommon.

 

Symptoms:

Left Heart Valve Disease 

Systolic murmurs:

Aortic stenosis: may be asymptomatic initially

-chest pain (increased O2 demands of hypertrophied ventricles), exertional dyspnoea, syncope/dizziness (on exercise)

(SAD = Syncope + Angina + Dyspnoea)

-Heart failure (usually after age 60) → dyspnoea

-Sudden death

Mitral regurgitation:

Acute MR may present with symptoms of left ventricular failure (as a cause of functional MR due to left ventricle dilation):

– dyspnoea, orthopnoea

Chronic MR can be asymptomatic or may present with:

-exertional dyspnoea, fatigue, palpitations (if AF)

Mitral prolapse: asymptomatic or atypical chest pain and palpitations

Some patients have symptoms of autonomic dysfunction- anxiety, panic attacks, syncope

Diastolic murmurs:

Aortic regurgitation: chronic AR initially asymptomatic

Later, symptoms of heart failure:

-exertional dyspnoea, orthopnoea, paroxysmal nocturnal dyspnoea, fatigue.

-occasionally, angina and palpitations

-syncope

Severe acute AR:

-sudden cardiovascular collapse

Symptoms related to aetiology e.g. chest or back pain in aortic dissection

Mitral stenosis: may be asymptomatic

-exertional dyspnoea, orthopnoea,fatigue, palpitations (if AF), chest pain

Rare:

-haemoptysis, cough- chronic bronchitis-like picture (backlog of blood to lungs)

+/- complications- hoarseness, dysphagia (compression by enlarged left atrium)

 

Right Heart Valve Disease 

Systolic murmurs:

Pulmonary stenosis: dyspnoea, fatigue, oedema, ascites (right ventricular failure). Can cause syncope.

Tricuspid regurgitation: 

-fatigue, breathlessness, palpitations

– hepatic (epigastric) pain on exertion, jaundice, ascites, oedema (lower limbs)

-headaches, nausea, anorexia

-dyspnoea and orthopnoea- if the cause is LV dysfunction

Diastolic murmurs:

Pulmonary regurgitation: usually no symptoms

Tricuspid stenosis: fatigue, ascites, oedema

 

Signs:

Left Heart Valve Disease 

Systolic murmurs:

Aortic stenosis:

-slow rising pulse

-narrow pulse pressure

parvus et tardus– feel for delayed and diminished carotid upstroke

-Heaving, non-displaced apex beat; left ventricular heave

-Aortic thrill (in aortic area, if severe)

Image result for ejection systolic murmur⇒Ejection systolic murmur– harsh crescendo- decrescendo murmur; heard at the base, left sternal edge and the aortic area

-radiates to the carotids and apex

-S1 usually normal.

A2 is quiet (S2) and may be inaudible in severe disease (calcified valve).

-As stenosis worsens, A2 is increasingly delayed leading to reversed splitting (P2 followed by A2- rare).

-There may be an ejection click (bicuspid valve) or an S4 (due to ventricular hypertrophy)

Mitral regurgitation:

-irregularly irregular pulse (if AF)

displaced, hyperdynamic, thrusting apex beat (left ventricular dilation- the more severe, the larger the left ventricle.)

-Right ventricular heave

-Signs of left ventricular failure in acute MR e.g. bibasal crackles due to pulmonary oedema

A pansystolic murmur associated with MRPansystolic murmur at apex, radiating to the axilla. Uniform intensity, merges with S2.

Soft S1

-Split S2, loud P2 (pulmonary hypertension)

-S3 may be heard (rapid ventricular filling in early diastole)

Mitral prolapse:

Mid-systolic click and/ or late systolic murmur

-The click moves towards S1 when standing (S1anding) and moves away on lying down.

Image result for murmur of mitral prolapse

Aortic regurgitation:

-collapsing (water hammer-pulse)

-wide pulse pressure

displaced, hyperdynamic- thrusting and heaving- apex beat (volume loaded)

An early diastolic murmur associated with AREarly diastolic murmur- high pitched, heard best in expiration with patient sitting forward, at lower left sternal edge.

-In severe AR, can get an Austin Flint murmur = mid-diastolic, low-pitched rumbling heard at the apex (due to fluttering of the anterior mitral valve cusp caused by the regurgitant stream, causing a physiological mitral stenosis)

-Weird signs named after people (associated with a hyperdynamic pulse):

Corrigan’s sign = visible carotid pulsation

de Musset’s sign = head nodding with each heart beat (Dad, can I borrow your car? Was that a yes? Yep! Thanks!)

Quincke’s sign = visible capillary pulsations in nail beds

Duroziez’s sign =  to and fro diastolic murmur heard when compressing the femorals proximally with the stethoscope

Traube’s sign = pistol shot sound over femoral arteries

Muller’s sign = visible pulsation of the uvula

Becker’s sign = visible pulsations of the pupils and retinal arteries

Rosenbach’s sign: systolic pulsations of the liver

Gerhard’s sign: systolic pulsations of the spleen

These are pretty much rare signs, but if there is an organ left over let me find a pulsation and have a sign in my name.

Mitral stenosis:

-malar flush on cheeks (due to low cardiac output = facial cyanosis)     -peripheral cyanosis

-low volume, thready pulse (thready = small fine pulse, feeling like a small cord or thread under the finge)

-atrial fibrillation common = irregularly irregular pulse

-tapping, non-displaced apex beat (palpable S1)

-parasternal heave (right ventricular hypertophy and pulmonary hypertension)

-evidence of pulmonary oedema (bibasal crackles), if decompensated

A mid diastolic murmur associated with MSRumbling, mid-diastolic murmur– low pitched, heard best in expiration with patient lying on left side.

Listen with bell of stethoscope to hear low pitched sound.

Loud S1, opening snap

-Graham Steell murmur may occur = early diastolic murmur (if there is pulmonary regurgitation secondary to pulmonary hypertension resulting from mitral stenosis)

 

Right Heart Valve Disease 

Systolic murmurs:

Pulmonary stenosis: prominent a wave in JVP

right ventricular heave

dysmorphic face (congenital cause)

Ejection systolic murmur, which radiates to the left shoulder

ejection click

-widely split S2

In severe stenosis

-murmur becomes longer and obscures A2

-P2 becomes softer and may be inaudible

-may hear S4

Tricuspid regurgitation:

-pulse may be irregularly irregular if AF (may occur with right atrial enlargement)

-raised JVP with giant v waves, which may oscillate the ear lobe, and prominent y descent in JVP

-Right ventricular heave/ parasternal heave

-Pulsatile hepatomegaly (tender, smooth, pulsatile)

-Jaundice

-Ascites

-Pitting oedema

-Chest signs of pleural effusion or causes of pulmonary hypertension (e.g. COPD)

Pansystolic murmur, heard best at lower left sternal edge in inspiration

-Loud P2 component of S2

Diastolic murmurs:

Pulmonary regurgitation:

Early decrescendo diastolic murmur, heard at the left sternal edge.

Called a Graham Steell murmur if the pulmonary regurgitation is secondary to pulmonary hypertension resulting from mitral stenosis.

Tricuspid stenosis: giant a wave and slow y descent in JVP

atrial fibrillation can occur

Early diastolic murmur, heard at the left sternal edge in inspiration

opening snap

 

Investigations:

ECG-

Left Heart Valve Disease 

Systolic murmurs:

Aortic stenosis:

  • P-mitrale (widened P wave with a notch near its peak = left atrial enlargement)

Image result for p mitrale

  • Left ventricular hypertrophy with strain pattern (deep S wave in V1-2, tall R wave in V5-6; inverted T waves in I, aVL, V5-6).
  • Left axis deviation (left anterior hemiblock/ left ventricular hypertrophy)
  • Poor R wave progression (= absence of normal increase in size of the R wave in the chest leads, advancing from V1 to V6. R wave height ≤ 3 mm in V3. Occurs in left ventricular hypertrophy.)

Image result for poor r wave progression

  • Left bundle branch block or complete AV block

 

Mitral regurgitation:

  • Atrial fibrillation
  • +/- P mitrale if in sinus rhythm (= delayed activation of left atrium due to left atrial enlargement)
  • Left ventricular hypertrophy

Mitral prolapse:

  • May show inferior T wave inversion

 

Diastolic murmurs:

Aortic regurgitation:

  • Left ventricular hypertrophy with strain pattern (deep S wave in V1-2, tall R wave in V5-6; inverted T waves in I, aVL, V5-6, left axis deviation).
  • Image result for left ventricular hypertrophy

(Voltage criteria = S wave in V1 + R wave in V5 or V6 (whichever is larger) ≥ 7 large squares)

Mitral stenosis:

  • Atrial fibrillation
  • P mitrale if in sinus rhythm (broad bifid p wave)
  • Right ventricular hypertrophy, in cases of severe pulmonary hypertension

Right Ventricular Hypertrophy = Right Axis Deviation, Dominant R waves in V1 + Dominant S waves in V6.

  • Progressive right axis deviation (right ventricular hypertrophy)

 

Right Heart Valve Disease

Pulmonary stenosis:

  • Right axis deviation
  • P pulmonale- peaked P wave, indicated right atrial hypertrophy (mitrale¿)
  • Right ventricular hypertrophy
  • Right bundle branch block

Tricuspid regurgitation:

  • P pulmonale- peaked P wave, indicated right atrial hypertrophy Image result for p pulmonale

Pulmonary regurgitation:

  • Right ventricular hypertrophy
  • Right bundle branch block

Tricuspid stenosis:

  • P pulmonale- peaked P wave, indicated right atrial hypertrophy

 

CXR-

Left Heart Valve Disease 

Aortic stenosis: left ventricular hypertrophy, calcified aortic valve, post-stenotic dilation of ascending aorta

Mitral regurgitation:

-Acute MR may produce signs of left ventricular failure e.g. pulmonay oedema

-Chronic MR shows enlargement of left atrium and left ventricle dilation (cardiomegaly), mitral valve calcification

Aortic regurgitation: cardiomegaly, dilated ascending aorta, pulmonary oedema (if left heart failure)

Mitral stenosis: left atrial enlargement (double shadow in right cardiac silhouette), cardiac enlargement, pulmonary oedema, mitral valve calcification

Right Heart Valve Disease 

Pulmonary stenosis: prominent main, right or left pulmonary arteries caused by post-stenotic dilatation.

Tricuspid regurgitation: right sided enlargement of cardiac shadow

 

Echo-

Doppler Echo is used to measure pressure gradients across valves and valve orifice areas in stenotic lesions. Detecting valvular regurgitation and estimating its significance is less accurate.

Aortic stenosis: diagnostic. Severe stenosis if peak gradient 50 mmHg and valve area < 1cm2. If the aortic jet velocity is > 4m/s risk of complications is increased.

Mitral regurgitation: to assess left ventricular function and aetiology (every 6-12 months for mod-severe MR to assess LV ejection fraction and end-systolic dimension). Assess suitability for repair rather than replacement. Doppler echo can assess size and site of regurgitation jet.

Mitral prolapse: diagnostic.

Aortic regurgitation: diagnostic. May indicate underlying cause e.g. aortic root dilation, bicuspid aortic valve, or the effects of AR (left ventricular dilation/ dysfunction and fluttering of the anterior mitral valve leaflet).

Mitral stenosis: diagnostic. Significant stenosis exists if the valve orifice is < 1cm2/m2 body surface area. Normal valve orifice area is ~4-6 cm2 and symptoms usually start when the orifice becomes < 2 cm2.

Echo is similarly diagnostic for right heart valve disease.

Cardiac catheter-

Can confirm diagnosis, assess valve gradient and left ventricular function- but risks emboli. Can assess severity of lesions and anatomy of aortic root, and also investigate coronary artery disease e.g. as a differential/ concomitant cause of angina.

Management:

-Treat any underlying cause.

-May give infective endocarditis prophylaxis for GI/GU infected procedures and oral penicillin as prophylaxis against rheumatic fever.

Left Heart Valve Disease

Aortic stenosis: if symptomatic prompt valve replacement recommended. If asymptomatic with severe AS and a deteriorating ECG, valve replacement also recommended. If patient not fit for surgical valve replacement, percutaneous valvuloplasty/ replacement (TAVI = Transcatheter Aortic Valve Implantation) may be attempted.

Manage left ventricular failure- ACE inhibitors and vasodilators should be used very cautiously in AS.

Mitral regurgitation: 

Diuretics can help with symptoms.

Control rate if in fast AF, and anticoagulate with warfarin.

Also anticoagulate if there is a history of embolism, prosthetic valve, or additional mitral stenosis.

If detoriating symptoms, surgery is indicated to repair or replace the valve before left ventricle is irreversibly impaired.

Mitral prolapse: beta blockers may help palpitations and chest pain.

In case of severe mitral regurgitation, surgery is needed.

Aortic regurgitation: the main goal of medical therapy is to reduce systolic hypertension. ACE inhibitors are helpful.

Echo every 6 months to monitor.

Indications for surgery: increasing symptoms, enlarging heart on CXR/echo, worsening left ventricle function (ejection fraction <50%), ECG deterioration (T wave inversion in lateral leads), infective endocarditis refractory to medical therapy.

Aim to replace valve before left ventricular dysfunction occurs- may be by open surgery or TAVI.

Mitral stenosis: 

Diuretics to reduce preload and pulmonary venous congestion.

If in atrial fibrillation, rate control, and anticoagulate with warfarin.

If medical management does not control symptoms: balloon valvuloplasty (if pliable valve = a balloon catheter inserted across the valve and inflated), open mitral valvotomy (= cutting through stenosed valve to relieve the constriction) or valve replacement.

 

Right Heart Valve Disease

Pulmonary stenosis: pulmonary valvuloplasty (widen with expanded ballon) or valvotomy (widen with cuts)

Tricuspid regurgitation: in functional TR medical therapy will improve signs and symptoms-diuretics, digoxin, ACE-inhibitors. Valve repair or replacement.

Pulmonary regurgitation: treatment rarely required

Tricuspid stenosis: diuretics, surgical repair.

 

Complications:

-Infective endocarditis, systemic emboli

-Heart failure

Aortic stenosis:

-Arrhythmias

-Stokes-Adam attacks

-MI

Left ventricular failure

-Sudden death

Mitral regurgitation:

-Left atrial enlargement

-AF (and resultant systemic emboli)

-Pulmonary oedema

-Pulmonary hypertension

Right heart failure

Mitral prolapse:

-mitral regurgitation

-cerebral emboli

-arrhythmias

-sudden death

Aortic regurgitation:

-Left ventricle dilation (caused by reflux of blood into the LV during diastole)

-Left ventricular failure

-Pulmonary oedema

Mitral stenosis:

-AF and systemic emboli

-pulmonary oedema

pulmonary hypertension and right heart failure

-pressure from left atrium on local structures: hoarseness (recurrent laryngeal nerve), dysphagia (oesophagus), bronchial obstruction

Tricuspid regurgitation:
heart failure

-hepatic fibrosis

Prognosis:

-Depends on aetiology, severity and valve.

e.g.

Aortic stenosis: if symptomatic, prognosis poor without surgery: 2-3 year survival if angina/syncope, 1-2 year if cardiac failure. And if mod-severe and treated medically, mortality up to 50% at 2 years.

Aortic regurgitation: poor post-op survival if ejection fraction < 50% or duration of congestive cardiac failure > 12 months.

Tricuspid regurgitation: ~10% 30 day mortality following valve replacement. TR resulting from myocardial dysfunction or dilatation has up to 50% 5 year mortality.

References: Cheese & Onion, Kumar and Clarke’s, Rapid Medicine, Lifeinthefastlane.com, fastbleep.com

 

 

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