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VALVULAR HEART DISEASE
Drawing of the Heart and its Blood Vessels, from the Anatomical Notebooks, Leonardo
da Vinci, Bibliotecha Ambrosiana, Milan, Italy, Late Fifteenth Century.
VALVULAR HEART DISEASE INDEX
1.
Aortic Incompetence.
2.
Aortic Stenosis.
3.
Mitral Incompetence.
4.
Mitral Stenosis.
5.
Mitral Valve Prolapse
6.
Pulmonary Incompetence.
7.
Pulmonary Stenosis.
8.
Tricuspid Incompetence.
9.
Tricuspid Stenosis.
Appendix 1:
Grading of the loudness of murmurs.
Appendix 2:
Auscultatory regions of the heart.
Appendix 3:
The JVP wave form.
VALVULAR HEART DISEASE AORTIC INCOMPETENCE
These guidelines are primarily based on the recommendations of The Task Force on
the Management of Valvular Heart Disease of the European Society of Cardiology,
2007. 1
Introduction
Aortic incompetence (AI) may be the consequence of diverse aetiologies, the distribution
of which has changed over time. The most frequent causes of AI are now those related to
aortic root disease and bicuspid aortic valve.
Natural History
Patients with acute AI have a poor prognosis without surgical intervention.
There is little information in the literature on the progression from mild to severe AI.
Patients with severe AI and symptoms have a poor prognosis.
The natural history of aortic root aneurysm has been mainly studied in patients with
Marfan’s syndrome. The strongest predictors of complication are the diameter of the
aortic root at the level of the sinuses of Valsalva and the presence of a family history of
cardiovascular events.
Causes
1.
2.
Valvular causes:
●
Infective endocarditis, (acute onset)
●
Rheumatic fever, (chronic onset)
Aortic root disruptions:
●
Marfans
●
Hypertension
●
Stanford type A dissecting aortic aneurysm.
●
Congential aortic root aneurysms.
●
Aortitis, (tertiary
arthropathies)
syphilis,
rheumatoid
arthritis,
sero-negative
Complications
1.
Infective endocarditis.
●
2.
Left heart failure:
●
3.
Note that aortic incompetence may also be caused by endocarditis, as well
as predisposing to it.
Left ventricular hypertrophy and eventually failure, the heart needs to
work against a volume load. In relative terms this is not as great as
working against a pressure load, (as for aortic stenosis).
Uncommonly angina pectoris, (possibly due to low diastolic pressures).
Clinical Features
In non acute cases:
Symptoms
1.
Initially a patient may be asymptomatic for years.
2.
Exertional dyspnea is generally the first symptom.
3.
Occasionally angina pectoris.
4.
Palpitations, related to a hyperdynamic circulation.
Signs
1.
2.
3.
Pulse:
●
Waterhammer, or “collapsing”.
●
Prominent carotid pulsations, (“Corrigan’s sign”)
Blood pressure:
●
Systolic pressure mildly increased
●
Diastolic pressure significantly reduced.
●
Increased pulse pressure.
Palpation:
Apex beat:
●
Hyperkinetic.
●
Somewhat displaced.
Thrill:
●
4.
Heart sounds:
●
5.
Occasionally detected at the left sternal edge, on expiration.
Soft 2nd heart sound (A2)
Murmur:
●
Early decrescendo diastolic murmur.
●
Maximal at the left sternal edge with valvular lesions, (right sternal edge
with root lesions)
●
Accentuated by sitting up and leaning forward with the breath held in
expiration.
●
May have an Austin Flint murmur, a diastolic murmur at the apex (sounds
like M.S but no opening snap and S1 is soft. It is due to the regurgitant
stream interfering with the anterior leaflet of the mitral valve during
diastole).
Clinical Indicators of Severity
SIGN
SEVERITY INDICATOR
Pulse
Collapsing nature and reflected in the blood pressure as a wide
pulse pressure.
Heart sounds
S3 (left ventricle) and soft A2
Murmur
Length of the murmur / presence of an Austin Flint murmur.
Heart failure
Left ventricular failure (late)
Investigations
CXR
Check for:
●
Cardiomegaly
●
Signs of pulmonary congestion.
ECG
●
Check for signs of LHV and strain.
Echocardiography
Indications of severe AI on echocardiography include: 1
●
A central jet with ≥ 65% of the LV outflow tract.
●
Moderate to severe LV enlargement in the absence of other aetiologies of LV
enlargement.
●
Regurgitant volume > 60 mls per beat.
●
Regurgitant fraction ≥ 50%
●
Effective regurgitant orifice area ≥ 0.3 cm2
Coronary angiography:
Coronary angiography is indicated in selected cases to detect associated coronary artery
disease when surgery is planned. Knowledge of coronary anatomy improves riskstratification and determines whether coronary revascularization is indicated in
association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical Therapy:
●
In patients with chronic severe AI and heart failure, ACE-inhibitors are the
treatment of choice when surgery is contraindicated or in cases with
persistent postoperative LV dysfunction.
●
3.
Surgical Therapy:
●
4.
In patients with Marfan’s syndrome, beta-blockers slow the progression of
the aortic dilatation and should also be given after operation. In patients
with severe AI, the use of beta-blockers should be very cautious because
the lengthening of diastole increases the regurgitant volume. However,
they can be used in patients with severe LV dysfunction.
The approach to the need for surgery can be summarized by the flow chart
below.
Screening:
●
In patients with Marfan’s syndrome or in young patients with aortic root
aneurysm, the family needs to be screened to detect asymptomatic cases.
VALVULAR HEART DISEASE AORTIC STENOSIS
These guidelines are primarily based on the recommendations of The Task Force on
the Management of Valvular Heart Disease of the European Society of Cardiology,
2007. 1
Introduction
Aortic stenosis (AS) has become the most frequent type of valvular heart disease in
Europe and North America. It primarily presents as calcific AS in adults of advanced age.
The second most frequent etiology, which dominates in the younger age group, is
congenital, whereas rheumatic AS has now become rare.
Natural History
Calcific AS is a chronic progressive disease. Patients typically remain asymptomatic
during long latent periods.
Sudden cardiac death is a frequent cause of death in symptomatic patients but appears to
be rare in the asymptomatic
As soon as symptoms occur, the prognosis is dismal and mortality has been reported to be
quite significant even within months of symptom onset which is often not promptly
reported by patients.
Causes
1.
Degenerative calcific aortic valve, (most common cause in adults).
2.
Calcified congenital bicuspid valve.
3.
Rheumatic fever, (early adulthood)
Complications
1.
Excertional syncope.
2.
Angina pectoris.
3.
Infective endocarditis.
4.
LVF, (late).
5
Sudden death.
Clinical Features
Symptoms
1.
Initially a patient may be asymptomatic for years as the cardiac output is
maintained by left ventricular hypertrophy.
Once symptoms do occur, average life expectancy will be less than 5 years if
untreated.
2.
Exertional dyspnea is generally the first symptom.
3.
Next symptoms to appear will be:
4.
●
Angina pectoris, (this is not related to coronary artery disease)
●
Exertional syncope
●
Sudden death.
Eventually signs of congestive cardiac failure, (this will indicate advanced disease
with average survival time of less than 2 years)
Signs
1.
Pulse (carotid)
●
2.
3.
Slow rising, sustained (or “plateau”) and small volume.
Blood pressure:
●
Reduced systolic pressure
●
Reduced pulse pressure
Palpation:
Apex beat:
●
Hyperdynamic, maybe slightly displaced.
Thrill:
●
4.
At the base of the heart, in the aortic area), if severe.
Heart sounds:
●
S4
5.
●
Splitting of the second heart sound, (ie paradoxical splitting on
expiration), if severe, (there is delayed left ventricular ejection and aortic
valve closure)
●
Soft or absent S2.
●
Occasionally an early systolic ejection click, (just after the first heart
sound). This will mean that the valve is mobile.
Murmur:
●
Harsh mid-systolic ejection murmur.
●
Maximal in the 2nd right intercostal space and radiates to the carotids
arteries.
●
Accentuated by sitting up and leaning forward with the breath held in
expiration.
Clinical Indicators of Severity
SIGN
SEVERITY INDICATOR
Pulse
Plateau, also reflected in the BP, Reduced systolic and pulse
pressures.
Heart sounds
S4, and paradoxical split of S2
Murmur
Long and late peaking, +/- an associated thrill.
Heart failure
Left ventricular failure is a very late sign.
Investigations
CXR
Check for:
●
Cardiomegaly
●
Signs of pulmonary congestion.
●
Aortic valve calcification may be seen.
ECG
●
Check for signs of LHV and strain.
Echocardiography
Indications of the severity of AS on echocardiography include: 1
●
Severe: Valve area of < 1cm2 or < 0.6 cm2/ m2 BSA.
●
Moderate: Valve area of 1.0-1.5 cm2 (0.6 cm2/m2 to 0.9 cm2/m2 BSA) or mean
aortic gradient 30–50 mmHg in the presence of normal flow conditions.
●
The combination of a markedly calcified valve (particularly without movement)
with a rapid increase in peak aortic velocity of 0.3 m/s per year.
●
AS with low flow gradients (<40 mmHg) and LV dysfunction with or without
contractile reserve.
Coronary angiography:
●
Coronary angiography is indicated in selected cases to detect associated coronary
artery disease when surgery is planned. Knowledge of coronary anatomy
improves risk-stratification and determines whether coronary revascularization is
indicated in association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical therapy:
●
The progression of degenerative AS is an active process sharing a number
of similarities with atherosclerosis. Thus, modification of atherosclerotic
risk factors must be strongly recommended following the guidelines of
secondary prevention in atherosclerosis.
●
Symptomatic patients require early surgery, as no medical therapy for AS
is able to delay the inevitability of surgery.
●
However, patients who are unsuitable candidates for surgery may be
treated with digitalis, diuretics, ACE-inhibitors, or angiotensin receptor
blockers if they are experiencing heart failure.
●
Beta-blockers should be avoided in AS.
●
3.
Co-existing hypertension should be treated; however, treatment should be
carefully titrated to avoid hypotension and patients more frequently
evaluated.
Surgical Therapy:
●
Aortic valve replacement is the definitive therapy for severe AS.
VALVULAR HEART DISEASE MITRAL INCOMPETENCE
These guidelines are primarily based on the recommendations of The Task Force on
the Management of Valvular Heart Disease of the European Society of Cardiology,
2007. 1
Introduction
Mitral incompetence (MI) is now the second most frequent valve disease after AS.
The treatment has been re-orientated as a result of the good results of valve repair.
Causes can be grouped into organic, ischemic and functional and within these groups
acute or chronic disease may occur.
Organic MI covers all aetiologies in which leaflet abnormality is the primary cause of the
disease, in opposition to ischaemic and functional MR, in which MR is the secondary
consequence of LV disease.
Natural History
Organic:
Acute MI is poorly tolerated and carries a poor prognosis in the absence of intervention.
In asymptomatic MI, the estimated 5 year rates of death from any cause, death from
cardiac causes, and cardiac events (death from cardiac causes, heart failure, or new AF)
with medical management are approximately 22%, 14%, and 33%, respectively.
Ischaemic:
Acute MI, secondary to papillary muscle rupture, has a dismal short-term prognosis and
requires urgent treatment.
Patients with chronic ischaemic MI have a poor prognosis.
Although coronary artery disease and LV dysfunction have prognostic importance, the
presence and severity of MI are independently associated with increased mortality.
Functional:
The data on the natural history and results of surgery are limited.
A precise analysis is difficult because of the limited number of series including small
numbers of patients and mixing patients with or without revascularization.
Several observational studies have shown the high prevalence of significant MI in
chronic heart failure, as well as its independent association with a poor prognosis.
However, its true prevalence and its pathogenic contribution to prognosis remain
uncertain.
Causes
1.
Organic:
Organic MI covers all aetiologies in which leaflet abnormality is the primary
cause of the disease.
These include:
2.
●
Degenerative, (most common).
●
Rheumatic fever.
●
Infective endocarditis.
●
Progression of mitral valve prolapse.
●
Connective tissue diseases, (including Marfans)
Ischaemic:
Ischaemic MI is a frequent entity, which is, however, frequently overlooked in the
setting of acute or chronic coronary disease.
3.
●
Chronic ischaemic MI may result from an ACS leading to papillary
muscle rupture or dysfunction.
●
Chronic ischaemic MI is the consequence of a restriction in leaflet motion,
which is due to tethering by the subvalvular apparatus in patients who
have LV enlargement and/or dysfunction, in particular of the
posterolateral wall.
Functional:
In this group, mitral valves are also structurally normal and MI is secondary to the
changes in LV geometry resulting from impaired LV function.
It includes MI seen in:
●
Cardiomyopathies
●
Ischaemic disease with severe LV dysfunction.
Complications
1.
Infective endocarditis.
●
Note that mitral incompetence may also be caused by endocarditis, as
well as predisposing to it.
2.
Left ventricular failure.
3.
Longer term, pulmonary hypertension with right ventricular failure.
Clinical Features
Symptoms
In acute lesions there will be rapid onset of pulmonary edema.
In the chronic setting there will be progressive dyspnea on exertion and features of LVF
in general
Signs
1.
Pulse:
●
2.
Blood pressure:
●
3.
4.
May be reduced in severe cases.
May be reduced in severe cases
Apex beat:
●
Displaced
●
Diffuse
●
Hyperdynamic.
Thrill:
●
May be detected in severe cases.
There may be a left parasternal heave if RVH/ pulmonary hypertension is present.
5.
Heart sounds:
●
Soft S1 if severe.
6.
Murmur:
●
Harsh pan systolic murmur best heard at the apex with radiation to the
axilla.
Clinical Indicators of Severity
SIGN
SEVERITY INDICATOR
Pulse
Small pulse volume.
Heart sounds
S3 and early diastolic flow rumble.
Murmur
Enlarged LV
Heart failure
Signs of pulmonary hypertension.
Investigations
CXR
Check for:
●
Cardiomegaly
●
Signs of pulmonary congestion.
ECG
●
Check for signs of LHV and strain.
Echocardiography
Indications of severe MI on echocardiography include: 1
●
Patients with severe LV dysfunction, LVEF< 30%, and/ or End systolic
dimension > 55 mm
●
Regurgitant volume > 60 mls per beat.
●
Regurgitant fraction > 50%
●
Effective regurgitant orifice area ≥ 0.4 cm2
Coronary angiography:
●
Coronary angiography is indicated in selected cases to detect associated coronary
artery disease when surgery is planned. Knowledge of coronary anatomy
improves risk-stratification and determines whether coronary revascularization is
indicated in association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical therapy (organic MI):
3.
●
In acute MI, reduction of filling pressures can be obtained with nitrates
and diuretics.
●
Nitroprusside reduces afterload and regurgitant fraction. Inotropic agents
should be added in case of hypotension.
●
Anticoagulant therapy, with a target international normalized ratio (INR)
range between 2 and 3, should be given in patients with MR and
permanent or paroxysmal AF or whenever there is a history of systemic
embolism or evidence of left atrial thrombus and during the first 3 months
following mitral valve repair.
●
In severe MI, maintenance of sinus rhythm after cardioversion is unlikely
unless the MI is treated surgically.
●
If AF occurs, heart rate should be controlled.
●
There is no evidence to support the use of vasodilators, including ACEinhibitors, in chronic MI without heart failure and therefore they are not
recommended in this group of patients.
●
On the other hand, when heart failure has developed, ACE-inhibitors have
a benefit and may be used in patients with advanced MR and severe
symptoms who are not suitable for surgery or when there are still residual
symptoms following the operation, usually as a result of impaired LV
function.
●
Beta-blockers and spironolactone should also be considered as
appropriate.
Surgical therapy:
The general approach to surgery in patients with severe organic MI is
summarized in the flow diagram below:
VALVULAR HEART DISEASE MITRAL STENOSIS
These guidelines are primarily based on the recommendations of The Task Force on
the Management of Valvular Heart Disease of the European Society of Cardiology,
2007. 1
Introduction
Although the prevalence of rheumatic fever has greatly decreased in industrialized
countries, mitral stenosis (MS) still results in significant morbidity and mortality
worldwide.
Since its development 20 years ago, percutaneous mitral (balloon) commissurotomy
(PMC) has impacted significantly upon the management of MS.
Natural History
Studies on natural history are old and non-controlled.
In asymptomatic patients, survival was good up to 10 years, progression being highly
variable with sudden deterioration, precipitated by complications, such as atrial
fibrillation or
embolism, in half of the patients.
Symptomatic patients have a poor prognosis.
Causes
1.
Rheumatic heart disease, (nearly always the cause)
Complications
1.
Infective endocarditis.
2.
Atrial fibrillation is common with the attendant risk of thrombo-embolic disease.
3.
Pulmonary edema.
4.
Pulmonary hypertension with consequent right ventricular failure.
5.
Recurrent chest infections.
Clinical Features
Symptoms
1.
Initially none, symptoms develop slowly.
2.
Essentially those of left heart failure, (although the left ventricle does not fail,
rather it is secondary to the increased left atrial pressures.
Therefore:
3.
4.
●
Exertional dyspnea.
●
Orthopnea.
●
Paroxysmal nocturnal dyspnea.
Chest:
●
Recurrent chest infections
●
Occasionally hemoptysis (ruptured bronchial veins)
Symptoms related to systemic emboli
Signs
1.
2.
Pulse
●
Small volume
●
AF is common.
Blood pressure
●
3.
May be reduced.
Palpation:
Apex beat
●
Tapping, (reflects a loud and palpable S1) Note, the term “tapping”
applies to MS, virtually by definition.
Thrill
●
4.
Heart sounds:
●
5.
Diastolic thrill at the apex, (very rare)
Loud S1
Murmur:
●
A low pitched mid-diastolic “rumble”, best heard with the bell lightly held
at the apex, with the patient leaning to the left side and breath held in
expiration.
May be accentuated by exercise.
●
Opening snap may precede the murmur (a sudden tensing of an
incompetently opened valve)
●
The murmur may continue on with a “pre-systolic accentuation”, due to
forceful left atrial systole, provided the patient is not in AF)
Therefore: H1 → H2 → Opening snap → rumbling mid-diastolic murmur
→ pre-systolic accentuation → H1
6.
Pulmonary hypertension, (late)
●
Left parasternal heave
●
Loud P2
●
Increased normal splitting of S2, (on inspiration)
●
Increased “a” wave of the JVP (if not in AF)
●
Pulmonary systolic murmur if there is associated PI, (rare)
Clinical Indicators of Severity
SIGN
SEVERITY INDICATOR
Pulse
Small pulse pressure
Heart sounds
Soft S1,( indicates immobile cusps, S1 is usually loud in MS)
Early opening snap, (due to increased left atrial pressure)
Murmur
Long diastolic murmur, (persists as long as there is a gradient)
Heart failure
Signs of pulmonary hypertension.
Investigations
CXR
Look for:
1.
Signs of pulmonary venous congestion.
2.
Signs of left atrial enlargement:
●
Double shadow behind the heart.
●
Widened carina (> 70 degrees)
●
Straightening of the left heart border.
●
On the lateral, posterior displacement of the esophagus.
●
On the lateral, a calcified valve, (which lies below the line joining the
carina to the xiphisternum)
ECG
Look for:
●
AF is common.
●
P mitrale, if in sinus rhythm.
●
Right ventricular hypertrophy / strain.
Echocardiography
Indications of the severity of MS on echocardiography include: 1
●
Valve area <1.5 cm2 or <1.7–1.8 cm2 in particular in cases of unusually large
patients.
Coronary angiography:
●
Coronary angiography is indicated in selected cases to detect associated coronary
artery disease when surgery is planned. Knowledge of coronary anatomy
improves risk-stratification and determines whether coronary revascularization is
indicated in association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medial therapy:
3.
●
Diuretics or long-acting nitrates transiently ameliorate dyspnoea.
●
Beta-blockers or heart-rate regulating calcium channel blockers are useful
to slow the heart rate in cases of rapid AF and can greatly improve
exercise tolerance by prolonging diastole and hence the time available for
LV filling via the stenosed valve.
●
Anticoagulant therapy with a target INR in the upper half of the range 2–3
is indicated in patients with either permanent or paroxysmal AF.
●
In patients with sinus rhythm, anticoagulation is mandatory when there
has been prior embolism or a thrombus is present in the left atrium, and
recommended when TEE shows dense spontaneous echo contrast or in
patients who have an enlarged left atrium (diameter >50 mm)
●
Cardioversion is not indicated before intervention in patients with severe
MS, as it does not usually restore sinus rhythm in the medium or long
term. If atrial fibrillation is of recent onset and the left atrium only
moderately enlarged, cardioversion should be performed soon after
successful intervention. Sinus rhythm can be maintained with the use of
class IC or III anti-arrhythmic drugs.
Surgical Therapy:
Decisions about surgical intervention are complex and will be determined by:
●
The severity of the MS
●
The severity of the patient’s symptoms
●
The age and co-morbidities of the patient
●
Echocardiographic hemodynamic parameters and predictors of PMC
success.
●
The expertise in a given procedure that is locally available.
Options include:
●
Percutaneous mitral (balloon) commissurotomy (PMC)
●
Open heart mitral commissurotomy.
●
Open heart surgery with mitral valve replacement.
General principles of the decision making process are represented in the flow
chart below:
VALVULAR HEART DISEASE MITRAL VALVE PROLAPSE
Introduction
Despite years of research, the symptomatology and significance of mitral valve prolapse
(MVP) remain controversial.
Once termed the “disease of the 1980s”, the great significance attached to it has since
been called into question. Initial studies that reported associated symptoms of MVP as
chest pain, dyspnea, anxiety, and panic, were probably flawed by recruitment bias.
Current thinking suggests that MVP could in most instances, simply reflect a normal
variant rather than a single disease process.
Recent studies further imply that the previously quoted incidence of MVP was
overestimated by inaccurate echocardiographic diagnostic criteria and that associated
symptoms, other than palpitations, are uncommon. 1
MVP is also commonly known as Barlow’s syndrome or Floppy valve syndrome.
Natural History
In general, primary MVP is a benign disorder with normal survival.
In a small number of cases secondary complications may occur as listed below.
Causes
Can be primary or secondary.
1.
Primary
●
MVP can be identified by echocardiography in 2-4% of the general
population and in most cases this finding simply reflects a normal
congenital variant rather than a specific disease process.
The MVP can be an inherited trait.
2.
Secondary causes:
In association with congenital conditions:
●
It may be seen in association with other congenital cardiac abnormalities.,
(Ebstein anomaly/ Atrial septal defect)
●
Connective tissue disorders, (Marfan syndrome and others)
●
Muscle disorders, (duchenne muscular dystrophy/ myotonic dystrophy)
Acquired:
●
MVP may be associated with IHD
●
Trauma
●
Rheumatic heart disease
Complications
Those with associated structural abnormalities (ie, thickened, deformed, or
redundant mitral valve leaflets) are more likely to suffer complications which may
include:
●
Endocarditis.
●
Stroke.
●
Progressive Mitral regurgitation.
●
Syncope/ Sudden death have been described.
Clinical Features
Primary MVP is often detected as an incidental finding.
Symptoms
The following have been described:
●
Atypical chest pain.
●
Palpitations.
●
Syncope.
Sudden death due presumed arrhythmia as also been reported however this is extremely
rare and in individual cases may not be related to the MVP at all.
Signs
1.
Pulse:
●
2.
There are no specific characteristics.
Blood pressure:
●
3.
Palpation
●
5.
There are no specific characteristics.
Heart sounds:
●
6.
There are no specific direct effects on the blood pressure.
There may be a mid to late systolic click (possibly due to a sudden tensing
of loose chordae tendinae as the leaflet prolapses) It has the characteristic
of a prosthetic valve rather than the softer quality of a normal heart sound.
Murmur:
●
There is a late systolic murmur best heard with the patient leaning to the
left with the breath held in expiration.
Notes:
●
Note that there may be a click only, a murmur only or a click and a murmur.
●
The clicks and murmurs may vary in intensity and hence may not be heard all the
time.
●
Manoeuvres which reduce LV volume (sudden standing and valsalva) cause the
click to come sooner (ie closer to the S1) and the murmur to become louder and
longer.
●
Manoeuvres which increase LV volume (squatting, passive leg raising, isometric
handgrip) prevent prolapse till late in systole and the click and murmur move
away from S1 toward S2
Investigations
CXR:
●
There are no specific features of note on CXR.
ECG:
●
There are no specific features of note on ECG
Echocardiography:
●
This will make the definitive diagnosis.
Coronary angiography:
●
Coronary angiography is indicated in selected cases to detect associated coronary
artery disease when surgery is planned. Knowledge of coronary anatomy
improves risk-stratification and determines whether coronary revascularization is
indicated in association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical therapy:
3.
●
Beta blockers have been advocated for troublesome symptoms due to
benign arrhythmias. These are best initiated by and after the assessment of
a cardiologist.
●
Aspirin or even warfarin may be required in some cases where there has
been a stroke or TIA or the patient develops AF. In most cases however
this will not be necessary.
Surgical therapy:
●
This will not be necessary in the majority of primary cases, unless
significant mitral incompetence develops. Careful observation by a
cardiologist over time will be all that is necessary.
●
In cases where the MVP is secondary to other cardiac disease the need for
surgical intervention will be more likely.
VALVULAR HEART DISEASE PULMONARY INCOMPETENCE
Introduction
Pulmonary incompetence is uncommon.
It is usually remarkably well tolerated when it does occur.
Natural History
Pulmonary incompetence of itself is often well tolerated and the overall natural history
and progress will more correlate with the underlying causative pathology.
Causes
1.
Chronic pulmonary hypertension from any cause.
These may include:
●
Primary pulmonary hypertension.
●
Cor pulmonale
●
Recurrent pulmonary embolism
Other causes are rare:
2.
Endocarditis.
3.
Rheumatic heart disease.
4.
Congenital lesions.
Complications
1.
Infective endocarditis.
●
2.
Note that pulmonary incompetence may also be caused by endocarditis,
as well as predisposing to it. But overall pulmonary valve endocarditis is
rare.
Right heart failure:
Clinical Features
The signs and symptoms will depend largely on the underlying pathology and its extent.
Symptoms
The symptoms of severe pulmonary incompetence/ pulmonary hypertension will include:
●
Dyspnea
●
Fatigue
●
Syncope
Signs
1.
Pulse:
●
2.
Blood pressure:
●
3.
May be reduced in severe disease.
May be reduced in severe disease.
Palpation:
Apex beat:
●
Not typically displaced.
●
There may be a right ventricular parasternal heave in cases of significant
right ventricular strain.
Thrill:
●
4.
Heart sounds:
●
5.
Not typically detected.
An S4 may be heard, (right ventricular strain)
Murmur:
●
High pitched blowing diastolic murmur best heard at the second left
intercostal space.
●
There may be an associated mid-systolic ejection flow murmur.
Investigations
CXR
Check for:
●
Cardiomegaly
●
Calcified pulmonary valve.
ECG
●
Check for signs of RHV and strain.
Echocardiography
●
This will confirm the diagnosis/ assess severity/ help determine the presence
pulmonary hypertension.
Coronary angiography:
Coronary angiography is indicated in selected cases to detect associated coronary artery
disease when surgery is planned. Knowledge of coronary anatomy improves riskstratification and determines whether coronary revascularization is indicated in
association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical Therapy:
●
3.
Diuretics may alleviate the symptoms of right heart failure.
Surgical Therapy:
●
Isolated pulmonary incompetence is well tolerated and surgical
intervention will only be necessary in severe cases.
VALVULAR HEART DISEASE PULMONARY STENOSIS
Introduction
Pulmonary stenosis (PS) is a rare condition.
It is usually seen in pediatric age groups as a congenital problem.
Natural History
Except for critical stenosis in neonates, survival is the rule in congenital PS.
The long-term course of patients with mild PS is usually benign. It does not tend to
progress in severity.
However, untreated severe PS may result in outflow obstruction that does progresses over
a period of years despite body growth, and will require intervention within 10 years of
diagnosis.
Causes
●
An isolated congenital condition.
●
As part of more complex congenital cardiac problem, such as Tetralogy of Fallot.
●
Subvalvular PS may also occur as a narrowing of the infundibular or
subinfundibular region, often with a normal pulmonic valve. This condition can
be associated with a ventricular septal defect.
Complications
1.
Infective endocarditis.
2.
Right heart failure
Clinical Features
Symptoms
Those with moderately to severe PS may experience exertional dyspnea and fatigue
Signs
1.
Pulse:
●
2.
May be reduced in severe cases.
Blood pressure:
●
3.
4.
Palpation
●
Right ventricular heave where there is RV hypertrophy
●
Thrill may be detected over the pulmonary valve in severe cases
Heart sounds:
●
5.
May be reduced in severe cases.
Murmur mat be preceded by an ejection click
Murmur:
●
Harsh systolic ejection murmur maximal at 2nd left intercostal space.
●
Murmur is increased by inspiration.
Investigations
CXR
Check for:
●
Cardiomegaly.
●
Calcified pulmonary valve.
ECG
●
Check for signs of RHV and strain.
●
RAD
Echocardiography
This will confirm the diagnosis and assess severity.
The pulmonary valve area of a healthy adult is 2.0 cm2/m2 of body surface area.
●
Mild valvular PS is defined by a valve area larger than 1 cm2 and a peak
transvalvular gradient of less than 50 mm Hg.
●
Moderately severe PS occurs if the valve area is 0.5-1.0 cm2, with a peak
transvalvular gradient between 50 and 75 mm Hg.
●
Severe PS is defined by a valve area smaller than 0.5 cm2 and a peak
transvalvular gradient of greater than 75 mm Hg.
Coronary angiography:
Coronary angiography is indicated in selected cases to detect associated coronary artery
disease when surgery is planned. Knowledge of coronary anatomy improves riskstratification and determines whether coronary revascularization is indicated in
association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical Therapy:
●
3.
Diuretics may alleviate the symptoms of right heart failure.
Surgical Therapy:
●
Balloon valvuloplasty is the preferred option provided the valve is
relatively compliant and mobile.
●
Those with severe valvular fibrocalcific thickening are more likely to
require a surgical approach.
VALVULAR HEART DISEASE TRICUSPID INCOMPETENCE
These guidelines are primarily based on the recommendations of The Task Force on
the Management of Valvular Heart Disease of the European Society of Cardiology,
2007. 1
Introduction
Minor degrees of tricuspid incompetence (TI) are commonly detected
echocardiography and may be termed normal “physiological” TI.
on
Like MI the pathological causes of TI can grouped into organic, ischemic and functional.
TI may also be associated with congenital heart disease.
Natural History
This will largely depend on the underlying pathology and associated pathologies.
Even severe TI may be well tolerated for a long period of time.
Functional TR may diminish or disappear as right ventricular failure improves following
the treatment of its cause. However, TR may persist even after successful correction of
left-sided lesions.
Causes
1.
Physiological:
●
2.
3.
Organic:
●
Bacterial endocarditis. This is nearly always seen in IV injecting drug
addicts.
●
Rheumatic fever. This is now rare in industrialized countries and is usually
seen in association with left sided valvular disease is seen, or mixed (ie
tricuspid stenosis and incompetence) disease.
Ischaemic:
●
4.
Minor degrees, very common echocardiographic finding.
Papillary muscle infarction, with dysfunction or rupture in the setting of
right ventricular infarction. This is much less common than ischaemic
induced MI.
Functional:
Functional TI is due to annular dilatation and is secondary to right ventricular
pressure and/or volume overload.
Pressure overload may be due to:
●
Pulmonary hypertension resulting from left sided heart disease.
●
Cor pulmonale, due to chronic lung disease.
●
Idiopathic pulmonary artery hypertension.
●
Dilated cardiomyopathies in general.
Volume overload may be due to:
5.
●
Atrial septal defects
●
Intrinsic disease of the right ventricle.
Congenital
●
Ebstein’s anomaly.
Complications
1.
Infective endocarditis
●
Note that tricuspid incompetence may also be caused by endocarditis, as
well as predisposing to it.
●
Infective endocarditis of the tricuspid valve is especially seen with
intravenous drug users.
2.
Right ventricular failure
3.
Cardiac cirrhosis (congestive hepatopathy)
Clinical Features
The signs and symptoms will predominantly relate to right sided heart failure.
Symptoms
These will primarily relate to cardiac failure.
Signs
1.
JVP
●
2.
Palpation:
●
3.
Elevated, often greatly so, with giant V waves, synchronous with the
carotid pulse.
Right ventricular heave on palpation.
Murmur:
A soft pan systolic murmur.
4.
5.
●
This is maximal at the left lower sternal edge.
●
It will increase on inspiration
●
Note that whilst this murmur is often difficult to hear the diagnosis of TI
can often be made on the other peripheral signs alone.
Abdomen:
●
Pulsatile hepatomegaly
●
Possibly some associated signs of hepatic congestion, such as ascites and
splenomegaly
●
Hepatojugular reflux, pressure over the liver may be seen to result in an
elevation of the JVP.
Legs:
●
Peripheral edema is common, and can be gross in chronic cases.
●
Signs of chronic venous insufficiency in long standing cases, (hemosiderin
deposition, varicosities, edema, venous eczema and ulceration)
Clinical Indicators of Severity
●
There are no well defined clinical parameters that correlate with severity.
Investigations
CXR
●
Right atrial enlargement may be seen.
ECG
●
P pulmonale
●
RVH and strain
Echocardiography
Indications of severe TI on echocardiography include: 1
●
Systolic reversal in the hepatic veins
●
IVC dilation and respiratory diameter variation < 50%
●
RA and RV dilation
Coronary angiography:
●
Coronary angiography is indicated in selected cases to detect associated coronary
artery disease when surgery is planned. Knowledge of coronary anatomy
improves risk-stratification and determines whether coronary revascularization is
indicated in association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical therapy
●
3.
Diuretics may improve signs of congestion, but attention to the underlying
condition is more important.
Surgical therapy
●
The timing of surgical intervention and the appropriate technique remain
controversial mostly due to the limited data available and their
heterogeneous nature.
●
As a general principle, and if technically possible, conservative surgery is
preferable to valve replacement, and surgery should be carried out early
enough to avoid irreversible right ventricular dysfunction.
●
The possible need for correction of TI is usually considered at the time of
surgical correction of left-sided valvular lesions.
VALVULAR HEART DISEASE TRICUSID STENOSIS
These guidelines are primarily based on the recommendations of The Task Force on
the Management of Valvular Heart Disease of the European Society of Cardiology,
2007. 1
Introduction
Tricuspid stenosis (TS) is almost exclusively of rheumatic origin.
It is rarely observed in developed countries, although it is still seen in developing
countries.
Detection requires careful evaluation, as it is almost always associated with left-sided
valve lesions that dominate the presentation.
Natural History
There is no good information on the natural history of isolated TS as the clinical picture
is usually dominated by the associated left sided lesions.
Causes
The cause is almost exclusively rheumatic fever.
Complications
1.
Infective endocarditis.
2.
Right heart failure.
Clinical Features
Symptoms
Will relate to those of right heart failure.
However the clinical picture is often overshadowed by features of associated left sided
heart valve lesions.
Signs
1.
Pulse:
●
2.
May be reduced in severe cases.
Blood pressure:
●
3.
Elevated JVP:
●
4.
5.
Giant a wave may be seen with a slow y descent.
Palpation:
●
Apex beat is not displaced.
●
Thrill is not an associated feature
Heart sounds:
●
6.
May be reduced in severe cases
There are no particular features of note.
Murmur:
●
A diastolic murmur audible at the left sternal edge, accentuated by
inspiration.
●
It is similar to the murmur of MS, except for the site of maximal intensity
and the effect of respiration, (louder on inspiration)
7.
The lesion is not associated with pulmonary hypertension.
8.
Abdomen:
●
Presystolic pulsation of the liver caused by forceful atrial systole may be
seen.
Clinical Indicators of Severity
These will relate to the signs of right sided heart failure.
Investigations
CXR
●
Right atrial enlargement
●
Calcified tricuspid valve.
ECG
●
Look for evidence of P pulmonale.
Echocardiography
●
No generally accepted grading of TS severity exists.
●
A mean gradient of > 5 mm Hg is considered indicative of clinically significant
TS.
Coronary angiography:
●
Coronary angiography is indicated in selected cases to detect associated coronary
artery disease when surgery is planned. Knowledge of coronary anatomy
improves risk-stratification and determines whether coronary revascularization is
indicated in association with valvular surgery.
Management
1.
Antibiotic prophylaxis for surgical procedures, (see latest edition of Antibiotic
guidelines)
2.
Medical therapy:
●
3.
In the presence of heart failure, diuretics are useful but of limited efficacy.
Surgical therapy:
●
Percutaneous balloon tricuspid dilatation has been performed in a limited
number of cases, either alone or alongside PMC, (for the mitral valve) but
frequently induces significant regurgitation. Data on evaluation of longterm results are lacking.
●
Intervention on the tricuspid valve is usually carried out at the time of
intervention on the other valves in patients who are symptomatic despite
medical therapy.
●
Conservative surgery or valve replacement, according to anatomy and
surgical expertise in valve repair, is preferred to balloon commissurotomy,
which can only be considered as a first approach in the rare cases of
isolated TS
Appendix 1
Grading of the loudness of murmurs:
Grade 1
Very soft, requires an experienced listener.
Grade 2
Soft.
Grade 3
Moderate and without a thrill
Grade 4
Loud with thrill just palpable.
Grade 5
Very loud and thrill easily palpable.
Grade 6
Very loud, may be heard without the aid of a stethoscope.
Appendix 2
Auscultatory regions of the heart:
Note, these regions show the optimal areas for listening to the heart valve indicated, they
do not exactly correlate with surface anatomy of the anatomical location of the valve.
Appendix 3
The JVP wave form:
Components of the jugular venous pressure wave with relationships to the first and
second heart sounds.
References
1.
Guidelines on the Management of Valvular Heart Disease. The Task Force on the
Management of Valvular Heart Disease of the European Society of Cardiology.
European Heart Journal January 2007 28: 230-268.
2.
Talley N.J, Clinical Examination 3rd ed 1996.
Dr. J.Hayes
4 October 2007