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VALVULAR HEART DISEASE
MUDr. Jan Tomis 5/2016
Normal valve function
•Maintain forward flow
and prevent reversal of
flow.
•Valves open and close
in response to pressure
differences (gradients)
between cardiac
chambers.
Abnormal valve function
•Valve Stenosis
•Obstruction to valve flow during that phase of the cardiac
cycle when the valve is normally open.
•Hemodynamic hallmark -“pressure gradient”
•Valve Regurgitation, insufficiency, incompetence
•Inadequate valve closure → reverse flow of the blood,
back leakage
•Combined – a single valve can be both stenotic and
regurgitant; combinations of valve lesions can coexist
•Single disease process
•Different disease processes
•One valve lesion may cause another
•Certain combinations are particularly common(AS & MR,
MS & TR)
Valvular heart disease
•Common cause of cardiovascular morbidity, 2nd most
common indication for heart surgery (after ischemic
heart disease)
• In the Czech Republic (2012): 8644 heart
surgeries, about 46% involved valve
intervention
• Combined myocardial revascularisation and
valve surgery particularly common for some
diagnosis – CABG + AVR (common risk factor),
CABG + MRV (in ischemic mitral regurgitation)
Diagnosis
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History
Physical examination
ECG
Chest x-ray
Echocardiography
Invasive evaluation, CT, MRI
Diagnosis
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History
Physical examination
ECG
Chest x-ray
Echocardiography
Invasive evaluation, CT, MRI
History
•Other known heart diseses
• Congenital heart disease,
bicuspid aortic valve
• Ischemic heart disease
• Rheumatic fever
• Hypertrophic/dilated
cardiomyopathy
• Aortic diseases – Marfans,
Ehlers-Danlos
History
• Generally: symptoms of
heart failure and low
cardiac output
• Breathlessness
• Chest pain or dyscomfort
• Syncope
• Fatigue
• Periferal or pulmonary
oedema
• Palpitations
Diagnosis
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History
Physical examination
ECG
Chest x-ray
Echocardiography
Invasive evaluation, CT, MRI
Physical examination
Physical examination
Physical examination
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MURMURS!!!
Periferal oedema
Lung crackles
Elevated JVP
Displaced apex beat,
irregular heart beat…
Hundreds of eponymous signs from past
millenium (↓importance in daily routine,
↑importance for passing exam)
Heart murmurs
•Sounds produces by turbulent
blood flow (in valve diseseses,
artery stenosis, abnormal chamber
or AV communication)
•Localization,grade,propagation,
timing, quality
Heart murmurs
Intensity
Description
Grade I/VI
Barely audible
Grade II/VI
Audible, but soft
Grade III/VI
Easily audible
Grade IV/VI
Easily audible, associated with a thrill
Grade V/VI
Easily audible, associated with a thrill, and still audible
with the stethoscope onlylightly on the chest
Grade VI/VI
Easily audible, associated with a thrill, and still audible
with the stethoscope off of the chest
Diagnosis
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History
Physical examination
ECG
Chest x-ray
Echocardiography
Invasive evaluation, CT, MRI
ECG
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Not specific
Findings might be caused or
altered by other concomitant
heart disease (hypertensive heart
disease, ischemic heart disease)
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Left ventricular hypertrophy
(aortic valve disease)
Left atrial enlargement (mainy
MS, but any left heart valve
disease)
Atrial fibrilation
Bundle branch block
Arrytmias (atrial fibrilation,
ectopic beats)
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Diagnosis
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History
Physical examination
ECG
Chest x-ray
Echocardiography
Invasive evaluation, CT, MRI
Chest x-ray in valvular disease
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Different heart shapes in different
valvular heart diseses, ↓specificity,
↓significance
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Cardiomegaly, pulmonary congestion
Widened mediastinum
Valve calcifications, prosthetic valves
Diagnosis
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History
Physical examination
ECG
Chest x-ray
Echocardiography
Invasive evaluation, CT, MRI
Echocardiography
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Mainstay of valve disease
diagnosis and follow-up
Allows real-time measurement
of chamber and wall diameters,
ejection fraction assessment
and functional valve evaluation
Easily avaiable and repeated
Essential in acute valve
disease diagnosis
No radiation harm
Trans-esophageal
echocardiography avaiable for
patients with poor transthoracic
sonographic window
Diagnosis
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History
Physical examination
ECG
Chest x-ray
Echocardiography
Invasive evaluation, CT, MRI
Invasive evaluation, CT, MRI
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Methods usualy used for uncertain cases or repeat cardiac
surgery / percutaneous inteventions planning
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Angiography to assess regurgitation severity – direct
transcatheter contrast medium administration into heart
chambers – aortography, ventriculography
Hemodynamic measurment – measuring of intracardial
pressures and gradients
CT aortography – method of choice in aortic dissection
diagnosis
CMRI – very precise evaluation of cardiac tissues and
function, but expensive, low avaiability, long examination time
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Aortic stenosis
•Most common indication for valve intervention
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Causes
• Degenerative aortic stenosis
• Bicuspid aortic valve
• Congenital aortic stenosis, unicuspid aortic valve
• Rheumatic disease (always with mitral valve involvement)
• Infective endocarditis (but severe stenosis due to massive
vegetations is extremely rare)
• Other rare causes – post radiation, associated with systemic
disease
Rheumatic
(involves commissures)
vs.
Degenerative
(spares commissures)
Aortic stenosis
•Pathophysiology
• Normal aortic valve area (AVA) – 3-4
cm2
• With a decrease of AVA ,a pressure
gradient develops between the left
ventricle and the aorta (increased
afterload)
• LV function initially maintained by
compensatory concentric hypertrophy
(but without an adequete increase in
vascularization)
• When compensatory mechanisms are
exhausted, LV function declines.
Aortic stenosis
Presentation
• Angina pectoris (increased myocardial
oxygen demand; demand/supply mismatch)
• Dyspnea on exertion due to heart failure
(systolic and diastolic)
• Syncope (exertional)
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Sudden death, mortality – when
asymptomatic with preserved left ventricle
ejction fraction, the sudden death risk is about
1%/y, when symptomatic, however, the
mortality increases to up to 50%/y
Aortic stenosis
Physical finding
• Systolic crescendo-decrescendo murmur with
maximum at right sternal border, 2nd-3rd
intercostal space , propagated to the carotic
arteries – the loundness of the murmur is not
directly correlated to severity of stenosis
• Slow rising carotid pulse (pulsus tardus) &
decreased pulse amplitude (pulsus parvus)
• Heart sounds - soft and split second heart
sound, S4 gallop due to LVH…
Aortic stenosis
Therapy – medical therapy has no prognostic effect
• Aortic valve replacement
• Standard therapy for patients with low surgical risk
or with indication for other procedure
• Mechanical/biological prosthesis
• TAVI (transcatheter aortic valve implantation) – patiens at
unaccaptable surgical risk (elderly, comorbid)
• Percutaneous aortic balloon valvuloplasty (for congenital
stenosis, or as a bridging therapy for unstable patients)
Aortic stenosis
Indication for replacement
• Severe aortic stenosis (AVA <1 cm2, mean PG > 40mmHg)
• Symptomatic
• LV function decreases
• Other indication for surgery
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Moderate stenosis (AVA 1,5-1 cm2 )
• With other indication for surgery
Aortic stenosis
Aortic regurgitation
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Causes
• Chronic aortic regurgitation
• Bicuspid aortic valve
• Rheumatic and degenerative – always with some
degree of stenosis
• Aortic root dilation (hypertension, Marfan’s, EhlersDanlos, syphylitic aortopathy)
• Other rare causes (SLE, RA)
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Acute aortic regurgiation
• Infective endocarditis
• Aortic regurgitation
Aortic regurgitation
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Pathophysiology of chronic aortic regrgitation
• Leakage of blood into LV during diastole due to
ineffective coaptation of the aortic cusps
• Combined pressure and volume overload
• Compensatory Mechanisms: LV dilation, LVH.
Progressive dilation leads to heart failure
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Greatest mass of myocardium in any valve disease –
„cor bovinum“ – over 500g
Aortic regurgitation
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Presentation
• Dyspnea: exertional, orthopnea, and
paroxsymal nocturnal dyspnea
• Chest pain
• Fatigue
• Palpitations: due to increased force of
contraction or arrytmias
Aortic regurgitation
•Physical findings (the ones you might find)
• Diastolic blowing murmur at the left sternal border –
might be very discrete. Systolic ejection murmur might be
present due to increased blood flow across the aortic
valve of concomitant valve stenosis
• Wide pulse pressure – caused by diastolic regurgitation
of blood to LV and fast decrease of diastolic BP –
„Corrigan’s pulse“ (160/30 mmHg…)
• Heaving and laterally displaced apex beat – due to
dilated heart with giant stroke volume
Aortic regurgitation
•Physical findings (the ones you might
not find…)
• Quincke’s sign - pulsations of nail
bed
• Muller’s sign - pulsation of uvula
• De Musset sign - (head nodding
in time with the heart beat)
• Duroziez sign (systolic and
diastolic murmurs heard over the
femoral artery when it is gradually
compressed with the stethoscope)
• Austin Flint murmur (apex):
Regurgitant jet impinges on
anterior MVL causing it to vibrate
Aortic regurgitation
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Acute aortic regurgitation
• Caused by a leaflet perforation in infective endocarditis
• In aortic dissection due to a change in aortic root
geometry – dilation, extensive intimal tear with prolapse
into LVOT and coaptation impairment
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Presentation of acute aortic regurgitation itself is usually a
pulmonary oedema accompanied by symptoms of the
causing pathology
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True emergency – mostly requires immediate cardiac
surgery
Aortic regurgitation
Therapy –surgical
• Isolated leaflet pathology - aortic valve replacement
• Aortic root pathology - combined aortic root, ascendent
aorta and aortic valve replacement – Bentall’s procedure
Aortic regurgitation
Indication for replacement
• Severe aortic regurgitation (EROA – effective regurgitant
orifice area >0,3 cm2)
• Symptomatic
• LV dilates (over 50 mm EDD) or function decreases (EF <
55%)
• Other indication for surgery
• Acute
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Moderate regurgitation (AVA 1,5-1 cm2 )
• With other indication for surgery
Mitral stenosis
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Causes
• Rheumatic heart disease in up to 99% of all
cases
• Other causes are rare - mitral annular
calcification, obstruction with massive
endocarditis vegetations, left atrial myxoma, post
radiation
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Nowadays rare in developed countries, still prevalent
in developing countries due to rheumatic fever
Mitral stenosis
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Pathophysiology:
• Normal mitral valve area 4-6 cm2 – stenosis
becomes severe with MVA < 1cm2
• Increased transmitral pressure gradient: leads to left
atrial pressure increase, enlargement and atrial
fibrillation →
• Development of postcapillary pulmonary hypertension
(there is no valve to isolate the increased left atrial
pressure from pulmonary veins) →
• Right heart failure symptoms - due to pulmonary HT,
secondary right ventricle dilation and tricuspid
regurgitation
Mitral stenosis
Presentation
• Dyspnea
• Syncope
• Palpitations (atrial fibrilation is common)
• In advanced cases - right heart failure symptoms – periferal
oedema, increased JVP, hepatomegaly, ascites…
Mitral stenosis
Physical finding
• Diastolic murmur
• Low-pitched diastolic rumble most prominent at the apex.
• Heard best with the patient lying on the left side in held
expiration
• Intensity of the diastolic murmur does not correlate with
the severity of the stenosis
• Lung crackles
• Pleural effusion
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Facies mitralis: When MS is severe and the cardiac output
is diminished, there is vasoconstriction, resulting in pinkishpurple patches on the cheeks (might be seen in terminal
heart failure of any cause)
Mitral stenosis
•Percutaneous therapy – PTMV –
in cases with suitable morphology
of mitral valve
•Surgical therapy -Mitral valve
replacement
•Medical therapy -Diuretics for
oedema, rate control therapy in
atrial fibrillation, anticoagulant
therapy (even in sinus rhytm with
great dilation of left atrium)
Mitral regurgitation
Causes
• Primary mitral regurgitation („valve is the pathology“) impairment of the valve itself, the cords or the papilary
muscles
• Myxomatous degeneration (Barlow valve disease)
• Leaflet prolapse (in Barlow disease or in normal valve
with cord rupture)
• Infective endocarditis
• Rheumatic valve disease
• Secondary mitral regurgitation („left ventricle it the
pathology“) – impairment of left ventricle function and
geometry
• Ischemic heart disease
• Dilated cardiomyopathy
• Hypertrophic cardiomyopathy
• Aortic valve disease
Mitral regurgitation
Pathophysiology
• Backflow of blood from the LV to the
LA during systole
• Pure volume overload – LV enddiastolic volume is increased by the
regurgitant volume
• Compensatory Mechanisms - left
atrial enlargement, LV increased
contractility and dilation
• Progressive left atrial dilation and
right ventricular dysfunction due to
pulmonary hypertension – in
advanced cases
Mitral regurgitation
Presentation
• Very long period of asymptomatic progresion
• Exertional dyspnea, fatigue
Physical finding
• Holosystolic murmur best heard at heart apex with
propagation to axilla
• In case of mitral leaflet prolaps, a systolic click might be
heard
Mitral regurgitation
Therapy
• Difers greatly according to the cause
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Primary
• Mitral valve repair – preserving the valve with surgically
correcting the cause of regurgitation – ruptured cords
replaced with artificial (Gore Tex) cords, resection of
redundant leaflet tissue
• Mitral valve replacement
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Secondary
• Treating the cause of ventricular dysfunction
• Medical, device therapy - in dilated CMP,
revascularization with annuloplasty in ischemic
heart disease
Mitral regurgitation
Acute mitral regurgitation
• Causes
• Cord rupture
• Infective endocarditis – leaflet perforation, cord rupture,
perivalvular fistula, absces
• Papillary muscle rupture (mechanical complication of
myocardial infarction, high force chest trauma)
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Presentation
• Acute heart failure – cardiogenic shock, pulmonary
oedema
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Management
• Diuretics, nitrates, IABC → emergency surgery
Tricuspid valve disesease
•Tricuspid regurgitation
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Is usually secondary – due to right ventricle dilation and failure as a result of
pumonary hypertension (the most common cause of right heart failure is left
heart failure) or volume overload (left-right shunt abnormalities)
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Cor pulmonale – right ventricle failure in pulmonary disease
•Primary:
•Infective endocarditis – in i.v. abusers
•Other are rare - Carcinoid, rheumatic, Ebstein anomaly – apical
displacement of septal and posterior leaflet of tricuspid valve →
„atrialization“ of a portion of the morphologic right ventricle
•Symptoms of right heart failure – periferal oedema, elevated JVP,
hepatomegaly, ascites…
•Symptoms treated by diuretics, surgical treatment is only indicated in case of
left sided valve intervention
Tricuspid valve disesease
Tricuspid valve disesease
•Tricuspid stenosis - rare
•Cause
•Rheumatic heart disease – never isolated
•Carcinoid valve disease, massive infective vegetation
Pulmonic valve disesease
•Isolated severe pulmonic valve diseases are extremely rare
•Pulmonic stenosis – congenital
•Systolic murmur at left sternal border, with possible
interscapulary propagation
•Pulmonic regurgitation – might be a result of pulmonary
hypertension, usually no treatment
Prosthetic heart valves