mitral stenosis (MS)

increased impedance or resistance to transmitral flow. MS attenuates the atrial

contribution to left ventricular filling and it also poses a hydraulic opposition or
resistance to early filling.

Rheumatic heart disease (RHD) is the most common cause of mitral stenosis (MS);
other causes are much less frequent The following are other causes of mitral stenosis:
Mitral annular calcificationRadiation-associated valve disease, including MS, is
increasingly recognized as late manifestation in survivors of Hodgkins lymphoma
Other rare conditions include Fabrys disease, Whipples disease,
mucopolysaccharidosis, methysergide therapy, carcinoid valve disease, endomyocardial
fibrosis, and systemic rheumatic disease (such as systemic lupus erythematosus and
rheumatoid arthritis).

Certain conditions produce hemodynamic abnormalities similar to those of native

valvular MS; these include atrial myxoma, large infected vegetations, ball valve
thrombus, and degenerated stenotic bioprosthetic mitral valve.

Chronic RHD affecting the mitral valve apparatus progresses over years to decades and
causes a number of pathologic changes, affecting the mitral valve apparatus

Hemodynamic and structural sequelae

The abnormal filling dynamics of MS are characterized by an increased transmitral

pressure gradient. This diastolic pressure gradient is dependent on the severity of MS,
cardiac output, and heart rate. In patients with mild to moderate MS, left atrial (LA)
pressures are only minimally elevated at rest, but may increase to produce symptoms
with exercise or other conditions that increase heart rate such as atrial fibrillation. In
more severe forms of MS, LA pressures are usually significantly elevated at rest. The
mean transmitral gradient is increased in patients with MS when the cardiac output
increases, as in anemic and pregnant patients . Progressive MS is associated with a
gradual loss of cross-sectional valve area. The normal mitral valve orifice has a cross-
sectional area of 4 to 6 cm2. In general, a valve area of <2.5 cm2 must be present before
exertional dyspnea can be attributed to MS. A valve area of <1.5 cm2 is usually required
to produce symptoms at rest. An orifice area of 1 cm2 is considered to represent severe
MS, and is associated with a significant pressure gradient

With isolated MS, the left ventricular systolic and diastolic pressures are usually
normal. However, when the stenosis is very severe, there may be a decrease in left
ventricular filling and end-diastolic volume (ie, preload), leading to reductions in stroke
volume and cardiac output. Furthermore, the chronically elevated LA pressure leads to
marked atrial remodeling and increased pulmonary venous, capillary, and arterial
pressures and resistance

Chronic pressure overload leads to a range of adaptive processes in the LA that

predispose to mechanical complications of LA chamber enlargement, in-situ
thrombosis, and atrial fibrillation. An enlarged LA may impinge on the left recurrent
laryngeal nerve, causing hoarseness (Ortners syndrome).
Pulmonary hypertension in patients with MS is the direct consequence of chronically
elevated pulmonary venous pressures. Pulmonary arterial hypertension eventually leads
to right ventricular hypertrophy and enlargement, tricuspid regurgitation, increased right
atrial pressure, and the development of right-sided heart failure

In the absence of correction of the stenotic lesion, rheumatic mitral stenosis (MS) is
generally a progressive disease. Development of clinical evidence of progression is slow
in asymptomatic patients but becomes more rapid after the onset of symptoms.The
duration of the asymptomatic phase of rheumatic MS varies across geographical areas].,
in North America, a latency period as long as 15 to 40 years between the initial
infection and the onset of clinical symptoms. In developing countries, on the other hand,
MS progresses much more rapidly, and may lead to symptoms in younger patients,
typically in young adults, or in the teenage years,

The prognosis dramatically worsens once the patient with MS develops symptoms. It
has been estimated that progression from mild symptoms to severe disability takes only
seven to nine years . Other poor prognostic indicators include the presence of atrial
fibrillation [46] and the progression to severe pulmonary hypertension

In addition to the gradual progression just described, as many as one-half of patients

have one or more episodes of acute deterioration due to one of the complications of MS
Chronic or paroxysmal atrial fibrillation occurs in approximately 45 percent of cases,
making it the most common cause of disability in a previously asymptomatic patient
Systemic thromboembolic events complicated MS in 13 to 26 percent of patients .The
majority of events occurred in patients with atrial fibrillation, but some had no known
history of this arrhythmia. Most of these emboli arose from the left atrium. Emboli can
also arise from the right atrium, leading to pulmonary embolism, when there is
pulmonary hypertension and right ventricular and atrial dilatation. MS and pulmonary
hypertension may develop functional tricuspid regurgitation .Death from MS is due to
progressive right-sided heart failure and/or pulmonary edema systemic thromboembolic
events or a large pulmonary
CLINICAL MANIFESTATIONS

Mitral stenosis (MS) usually presents with exertional dyspnea and/or decreased exercise
tolerance are primarily related to the severity of the valvular stenosis. Many patients
with severe MS do not recognize symptoms because slow progression of disease is
accompanied by a gradual reduction in activity.

Less common clinical presentations include hemoptysis, chest pain (often due to
pulmonary hypertension), fatigue (which may be associated with low forward flow and
a low transmitral gradient), ascites and lower extremity edema associated with right
heart failure ,stroke or other thromboembolic event (particularly in patients with atrial
fibrillation), hoarseness, and infective endocarditis

Chest pain Patients with MS rarely present with chest pain. Although the pain often
resembles angina and may be due to underlying coronary artery disease or a coronary
artery embolism, it is most commonly the result of pulmonary hypertension and right
ventricular hypertrophy. Another cause of intermittent chest pain is an atrial
tachyarrhythmia with left atrial and pulmonary vascular distension.

Chronic pulmonary hypertension eventually leads to increased right ventricular and

right atrial pressures, right ventricular enlargement, tricuspid regurgitation, and signs of
right-sided heart failure. Increased jugular venous p ressureLower extremity edema,
which may progress to involve the upper thighs, sacral area, and abdominal wall; ascites
and pleural effusions can also occur. Hepatomegaly

Hoarseness If the left atrium becomes very large, there may be compression of the
recurrent laryngeal nerve, leading to hoarseness (Ortner's syndrome or cardiovocal
syndrome) or coughing.

Any situation that increases the cardiac output, which raises transmitral flow, or causes
tachycardia, which decreases diastolic filling time, can markedly increase the
transmitral pressure gradient and precipitate symptoms . examples are exertion,
emotional stress, fever, pulmonary infection, AF, and pregnancy.

Cardiac auscultation first heart sound (S1) is loud, The second heart sound is initially
normal but, with the development of pulmonary hypertension, P2 becomes increased in
intensity and may be widely transmitted. As pressure increases further, splitting of S2 is
reduced and ultimately S2 becomes a single sound. Opening snap The OS is due to the
abrupt halt in leaflet motion in early diastole, after rapid initial rapid opening, due to
fusion at the leaflet tips. It is best heard at the apex and lower left sternal border. The
murmur caused by MS is a low-pitched diastolic rumble most prominent at the apex.

Stress (exercise or dobutamine) echocardiography is particularly helpful when

symptoms seem to be more severe than expected based on resting hemodynamics (such
as resting valve area); these patients often have exertional pulmonary hypertension that
can be detected on stress echocardiogram.)

DIFFERENTIAL DIAGNOSIS Left atrial myxoma Prosthetic valve obstruction (by

acute or chronic thrombus, pannus ingrowth, or leaflet calcification)
STAGING Stage A includes patients at risk for MS Stage B includes asymptomatic
patients with progressive MS. Stage C includes asymptomatic patients with severe MS
defined as a mitral valve area 1.5 cm2 Stage D, includes symptomatic severe MS
defined as a mitral valve area 1.5 cm2

The normal mitral valve orifice has a cross-sectional area of about 4.0 cm2. When the
orifice is reduced to 2 cm2, the pressure gradient across the valve begins to increase.
Many patients develop symptoms when the mitral valve area is reduced to 1.5 cm2 and
nearly all patients becoming symptomatic when valve area is reduced to 1.0 cm2 or less.

For all patients, the follow-up should include yearly history and physical examination.
echocardiography every three to five years if the mitral valve area (MVA) is >1.5 cm2,
every one to two years if the MVA is 1.0 to 1.5 cm2, and once per year if the MVA is
<1.0 cm2 as mitral valve area declines at a mean of about 0.1 cm2 per year. the rationale
for monitoring is to optimize timing of mitral valve intervention. The timing of surgical
or percutaneous intervention for MS is crucial for the following reasons:If performed
too early, the patient may be put at unnecessary risk for a complication of the procedure
with little or no short-term benefit because MS (in the absence of intervention) may
remain asymptomatic and stable for many years.Delaying intervention may cause
irreversible pulmonary hypertension and/or right heart failure.

In patients who develop indications for mitral valve intervention, the feasibility of
percutaneous mitral balloon valvotomy is assessed by using the Wilkins score , absence
of left atrial thrombus and lack of moderate to severe mitral regurgitation (MR).
Anicoagulation (with vitamin K antagonist INR 2.5) in patients with MS and one or
more of the following conditions: Atrial fibrillation (paroxysmal, persistent, or
permanent) Prior embolic event Left atrial thrombus

transesophageal echocardiographic in moderate to severe MS in sinus rhythm found

left atrial thrombus in 7 percent

Diuretic therapy (usually with a loop diuretic) and dietary salt restriction are appropriate
if manifestations of pulmonary vascular congestion or right heart failure

Beta blockers can significantly decrease heart rate and cardiac output at rest, causing a
decrease in the transmitral gradient, pulmonary venous pressure, and mean pulmonary
artery pressure in patients with MS. Beta blockers can blunt the heart rate and cardiac
output responses to exercise, while attenuating the rise in transmitral gradient that
normally occurs [

Atrial fib causes exaggerated adverse hemodynamic effects caused in MS and prompt
therapy is often necessary ,if unstable, immediate electrical cardioversion or rate
control (with a beta blocker, calcium channel blocker [verapamil or diltiazem], or, less
preferably, digoxin) and anticoagulation. When MS is hemodynamically significant
(valve area <1.5 cm2), flow across the mitral valve is maintained by two factors:
adequate diastolic filling time and higher-than-normal pulmonary venous pressure.

Patients with rheumatic MS should receive antibiotic prophylaxis for secondary

prevention of rheumatic fever

only patients with the highest risk of the development of endocarditis (eg, patients with
prosthetic heart valves, patients with prior endocarditis) are advised to receive
antimicrobial prophylaxis . Most patients with native valvular heart disease, including
those with mitral stenosis, are not included in this group and therefore do not require
antimicrobial prophylaxis.

Mortality improvement can only be achieved by intervention with percutaneous mitral

balloon valvotomy or surgery

percutaneous mitral balloon valvotomy for symptomatic patients with severe MS

(mitral valve area [MVA] 1.5 cm2, stage D) in patients with favorable valve
morphology, absence of left atrial thrombus, and absence of moderate to severe mitral
regurgitation (MR).

mitral valve surgery (repair, commissurotomy, or valve replacement) in severely

symptomatic patients (New York Heart Association [NYHA] class III or IV) with
severe MS (MVA 1.5 cm2, stage D) who are not high risk for surgery and who are not
candidates for or who have failed prior percutaneous mitral balloon valvotomy.

concomitant mitral valve surgery for patients with severe MS (MVA 1.5 cm2, stage
C or D) undergoing cardiac surgery for other indications.
 percutaneous mitral balloon valvotomy for asymptomatic patients with very severe
MS (mitral valve area 1.0 cm2, stage C), favorable valve morphology, absence of
moderate to severe MR, and no left atrial thrombus.

mitral valve surgery for severely symptomatic patients (NYHA class III to IV) with
severe MS (MVA 1.5 cm2, stage D) who are candidates for percutaneous intervention
but are undergoing surgery with cardiopulmonary bypass for other indications (eg,
aortic valve disease, coronary artery disease, tricuspid regurgitation, aortic aneurysm).

percutaneous mitral balloon valvotomy for selected asymptomatic patients with

severe MS (MVA 1.5 cm2, stage C) with new onset atrial fibrillation (AF) and
favorable valve morphology, absence of moderate to severe MR, and no left atrial
thrombus.

percutaneous mitral balloon valvotomy for selected symptomatic patients with

moderate MS MVA >1.5 cm2 if pulmonary artery wedge pressure is >25 mmHg or
mean mitral valve gradient is >15 mmHg during exercise and valve morphology is
favorable and there is no moderate to severe MR and no left atrial thrombus.

percutaneous mitral balloon valvotomy for selected severely symptomatic patients

(NYHA class III to IV) with severe MS (MVA 1.5 cm2, stage D) who have a
suboptimal valve anatomy and who are not candidates for surgery or at high risk for
surgery.

mitral valve surgery for selected patients with moderate MS (MVA 1.6 cm2 to 2.0
cm2) with mitral valve anatomy unfavorable for percutaneous intervention
(echocardiogram score and presence of MR) undergoing cardiac surgery for other
indications.

mitral valve surgery and excision of the left atrial appendage for selected patients with
severe MS (MVA 1.5 cm2, stage C and D) who have had recurrent embolic events
while receiving adequate anticoagulation.

Pre-operative evaluation MS who are asymptomatic and do not have pulmonary

hypertension or atrial fibrillation probably do not incur an excess risk in noncardiac
surgical procedures. In comparison, the prognosis is poor in patients with untreated
symptomatic MS. Pre-operative percutaneous balloon mitral valvotomy is suggested
for patients with asymptomatic severe MS who are candidates for percutaneous
intervention (including favorable valve anatomy) and undergoing elective moderate- or
high-risk noncardiac surgery.

Perioperative management of unoperated MS undergoing noncardiac surgery

Adequate heart rate control Tachycardia should be avoided . Among patients with
atrial fibrillation, the ventricular rate should be well controlled prior to elective
surgery.nCareful management of central blood volume Preload should be monitored
and maintained high enough to allow adequate cardiac output but low enough to avoid
pulmonary edema. Because of the obstruction to flow from left atrium to left ventricle,
any situation that increases venous return and central blood volume will lead to a rise in
pulmonary pressures and congestion. As an example, the Trendelenburg position
dramatically increases pulmonary blood flow and can cause overt pulmonary edema.
Right heart monitoring during surgery is essential to manage the volume status.
Maintenance of systemic vascular resistance MS impairs the normal compensatory
response associated with a fall in blood pressure, since the stroke volume cannot be
substantially increased. As a result, maintenance of systemic vascular resistance is
important, since sudden drug-induced reductions in systemic vascular resistance can
lead to hypotension. Avoidance of marked changes in pulmonary vascular resistance
Some degree of pulmonary hypertension is present in patients with MS. However, an
increase in pulmonary hypertension (eg, from arterial hypoxemia) may produce frank
right ventricular decompensation.

chronic mitral regurgitation

Mitral regurgitation (MR) may be due to a primary abnormality (sometimes referred to organic MR) of one or more
components of the valve apparatus (leaflets, chordae tendineae, papillary muscles, and/or annulus) or may be
secondary (previously referred to as functional MR) to another cardiac disease (such as coronary heart disease or a
cardiomyopathy)

The causes of primary MR include: Degenerative mitral valve disease (including mitral valve prolapse) is the most
common cause of primary MR Rheumatic heart disease. Rheumatic valve disease often results in MR in the first two
decades of life [1], while mitral stenosis and mixed mitral stenosis plus MR are more often seen in adults Infective
endocarditis. Trauma, Use of certain drugs, such as ergotamine, bromocriptine, pergolide, and cabergoline
Congenital Mitral annular . Causes of flail or partial flail mitral leaflet include mitral valve prolapse, infective
endocarditis, trauma, and rupture of a papillary muscle in the setting of an acute myocardial infarction.

secondary causes of MR Coronary heart disease, most often is due to a regional wall motion abnormality MR also
can occur with transient myocardial dysfunction so that MR severity (and symptoms) may acutely worsen with
ischemia, exercise, or other changes in hemodynamic conditions. Dilated cardiomyopathyHypertrophic
cardiomyopathy with MR due to abnormal systolic anterior motion of the mitral leaflets. Right ventricular pacing
can cause worsened or de novo secondary MR.

Symptoms severity of MR, its rate of progression, the pulmonary artery pressure, arrhythmias (eg, atrial fibrillation),
and associated cardiac disease. Because of the importance of identifying the transition from asymptomatic to
symptomatic MR in determining the timing of mitral valve surgery, a careful history is important to establish a good
estimate of baseline exercise tolerance Patients with isolated mild to moderate primary MR are asymptomatic, since
there is little volume overload of the ventricle and cardiac hemodynamics, and forward cardiac output remains
normal. Most remain asymptomatic until there is left ventricular cavity enlargement with systolic dysfunction,
pulmonary hypertension, or the onset of atrial fibrillation. The most common symptoms are exertional dyspnea and
fatigue due to the combination of a decreased forward (ie, effective) cardiac output, an increase in left atrial pressure
due to backflow across the mitral valve, and pulmonary artery hypertension.. Patients with severe MR and left
ventricular enlargement eventually progress to symptomatic heart failure with pulmonary congestion and edema. At
this stage of left ventricular dilatation, the myocardial dysfunction is often irreversible due to the long-standing MR.

Most patients with chronic secondary MR (ischemic or non-ischemic) have mild MR, and the clinical presentation
(including symptoms and signs of heart failure) generally reflects the state of ventricular dysfunction more than the
state of the mitral valve. However, patients with only mild secondary MR at rest may develop severe MR with
exercise, leading to acute pulmonary edema .The most common complaints reflect decreased forward or effective
cardiac output and include weakness, fatigue, and exercise intolerance. Patients with more severe disease or with
exercise-induced worsening of MR may present with symptomatic heart failure or pulmonary edema. However, it
may be difficult to separate symptoms due to primary left ventricular systolic dysfunction from those due to the
added burden of secondary MR.

Other symptoms such as thromboembolism, hemoptysis, and right-sided heart failure do occur, but are less common
than with MS- there is an increased risk of infective endocarditis in patients with an abnormal mitral valve and
moderate to severe MR.

Heart sounds S1 is diminished, reflecting failure of the mitral leaflets to close properly..With chronic MR, wide
splitting of S2 may be present, if pulmonary artery hypertension is present with a delay in P2. With acute MR,
splitting of S2 is present because the decrease in left ventricular ejection time results in an early A2.The augmented
flow rate across the mitral valve orifice into a dilated left ventricle produces an S3 gallop, which becomes particularly
prominent if left ventricular failure occurs.The murmur of MR is systolic ,immediately after S1 Infrequently, the
large diastolic volume across the mitral valve with severe MR produces an early diastolic murmur

The most common finding on the chest radiograph is cardiomegaly ECG left atrial enlargement

Mitral regurgitation (MR) should be suspected in patients with an apical holosystolic or mid to late systolic murmur
and confirmed by transthoracic echocardiogram .If MR severity and left ventricular systolic function cannot be
adequately assessed by TTE and TEE, cardiovascular magnetic resonance imaging is indicated. Stress testing
(combined with Doppler echocardiography or cardiac catheterization) is helpful in selected patients with chronic
primary MR to evaluate exercise-induced changes in hemodynamics. Stress testing (myocardial perfusion imaging or
stress echocardiography) may be used to identify ischemia in patients with suspected ischemic MR.

Identifying the severity of MR with calculation of regurgitant volume ,regurgitant orifice area, regurgitant fraction is
important Echocardiographic criteria for severe MR include vena contracta width 0.7 cm, regurgitant fraction 50
percent, and regurgitant jet area >40 percent of left atrial area or a holosystolic eccentric jet. Additional criteria for
severe MR include effective regurgitant orifice (ERO) area 0.40 cm2 and regurgitant volume 60 mL.

Primary MR is diagnosed when imaging demonstrates abnormal mitral valve leaflets or chordae. Secondary
(functional) MR due to left ventricular dilation and systolic dysfunction is characterized by failure of the mitral leaflets
to fully coapt and a dilated mitral valve annulus.

Assessment of MR should include effects on left atrial size, left ventricular size and function, and pulmonary artery
pressure. Left atrial size is usually increased. Left ventricular size and systolic function are normal early in the disease
course but progressive ventricular dilation and a decline in ejection fraction occur with chronic severe MR. Severe
chronic MR generally does not exist without left atrial or ventricular enlargement. If the left ventricular end-d.iastolic
dimension (by echocardiography) is less than 60 mm (approximately 35 mm/m2), the diagnosis of severe chronic MR
should be seriously questioned

evaluation of coronary anatomy by computed tomography (CT) or invasive angiography is indicated before valve
intervention in patients with suspected coronary artery disease (including symptoms of angina or objective evidence
of ischemia), decreased left ventricular systolic function, or coronary risk factors (including men >40 years old and
postmenopausal women) In patients undergoing cardiac catheterization for other indications, left ventriculography
may establish the presence and severity of MR. In contrast to its occasional utility in chronic MR, contrast left
ventriculography is generally avoided at the time of catheterization in patients with acute MR because of the
additional iodinated contrast load in an already compromised patient

Stress testing In patients with chronic MR with symptoms that seem out of proportion to severity of MR at rest,
measurement of supine bicycle, exercise-induced changes with either Doppler echocardiography (to assess changes
of MR and pulmonary artery pressure) or cardiac catheterization (to assess pulmonary capillary wedge pressure and
left ventricular diastolic pressure) may be helpful in selected cases but is not routinely recommended Among patients
with ischemic MR, exercise echocardiography may be helpful in patients with mild MR at rest. Some of these patients
have a marked increase in MR severity and pulmonary artery pressure with exercise,

The apical holosystolic murmur of mitral regurgitation (MR) is distinguished from aortic stenosis or hypertrophic
obstructive cardiomyopathy with left ventricular obstruction., tricuspid regurgitation causes a holosystolic murmur
that is located at the left lower sternal border and increases with inspiration. A ventricular septal defect causes a
harsh holosystolic murmur (often with a thrill) at the left sternal border

Treatment

In stage A, patients are at risk of MR (or have mild MR). mild mitral valve abnormalities (mild mitral valve prolapse or
mild valve thickening and leaflet restriction) with no or mild MR (small central jet <20 percent of left atrium on color
Doppler with vena contracta <0.3 cm). The left atrial and ventricular sizes are normal and there are no symptoms.

In stage B, patients have progressive MR with substantial valve abnormalities (severe mitral valve prolapse with
normal coaptation, rheumatic valve changes with leaflet restriction and loss of central coaptation, or evidence of
prior infective endocarditis).
Progressive MR is identified by the following criteria: a central jet filling 20 to 40 percent of the area of the left
atrium or late systolic eccentric MR, vena contracta of 0.3 to <0.7 cm, regurgitant volume <60 mL, regurgitant
fraction <50 percent, and effective regurgitant orifice is <0.40 cm2. There is mild left atrial enlargement, no left
ventricular enlargement, normal pulmonary pressure, and no symptoms.

In stage C, patients have asymptomatic severe MR. There are severe valve abnormalities (severe mitral valve
prolapse with loss of coaptation or flail leaflet, rheumatic valve changes with leaflet restriction and loss of central
coaptation, evidence of prior infective endocarditis, or thickening of leaflets with radiation heart disease). Severe
MR is identified by presence of at least some of the following criteria: a central jet of MR >40 percent of the left
atrium or holosystolic eccentric MR. Vena contracta is 0.7 cm, the regurgitant volume is 60 mL, the regurgitant
fraction is 50 percent, and the effective regurgitant orifice is 0.40 cm2. The angiographic grade is 3 to 4+.There is
moderate or severe left atrial enlargement, left ventricular enlargement, and pulmonary hypertension is present at
rest or with exercise.

There are two subgroups in stage C: -Patients with C1 disease have left ventricular ejection fraction >60 percent
and left ventricular end systolic dimension <40 percent. Such patients are said to have chronic compensated MR -
Patients with C2 disease have left ventricular ejection fraction 60 percent and left ventricular end systolic dimension
40 mm. Patients with C2 disease are exhibiting a transition to Stage D

In stage D, patients have symptomatic severe MR with severe valve abnormalities Severe MR is identified as
above.Moderate or severe left atrial enlargement, left ventricular enlargement, and pulmonary hypertension are
present. Symptoms include decreased exercise tolerance and exertional dyspnea or a history of overt heart failure.

Follow-up of all patients with valve disease includes at least annual history and physical examination. Patients with
mild MR and no evidence of left ventricular enlargement, left ventricular dysfunction, or pulmonary hypertension
.echocardiography every three to five years.- moderate MR echocardiography every one to two years.- severe MR
should be seen every 6 to 12 months

coronary angiography is recommended prior to mitral valve surgery in patients who have coronary disease or are at
risk for coronary disease

There is a limited role for medical management in patients with chronic primary mitral regurgitation (MR), since valve
intervention is required to address the primary process.

Symptomatic patients with chronic primary MR (stage D) and left ventricular ejection fraction (LVEF) <60 percent
who are awaiting valve surgery or who are not candidates for valve surgery are treated with standard medical
therapy for heart failure (including angiotensin converting enzyme [ACE] inhibitor [or angiotensin II receptor blocker
or angiotensin receptor-neprilysin inhibitor], beta blocker, aldosterone antagonist, and diuretics). Given lack of
evidence of efficacy, we do not use vasodilators to treat normotensive asymptomatic patients with chronic primary
MR (stages B and C1) and normal systolic LV function. Limited evidence on beta blocker therapy in patients with MR
suggests a possible benefit.

antibiotic prophylaxis is not recommended when patients with mitral valve disease (in the absence of prosthetic
valve replacement or history of infective endocarditis) undergo dental or other invasive procedures that produce
significant bacteremia. For patients with rheumatic heart disease, long-term prophylaxis against rheumatic fever is
recommended

The decision to proceed with mitral valve intervention depends upon the stage (including presence of symptoms, left
ventricular ejection fraction [LVEF], left ventricular end-systolic dimension [LVESD . When surgical treatment is
indicated for patients with chronic severe primary MR, when successful and durable mitral valve repair is expected,
mitral valve repair is preferred to mitral valve replacement

For patients with prohibitive surgical risk because of severe comorbidities who are severely symptomatic (New York
Heart Association [NYHA] class III to IV) with chronic severe primary MR (stage D) and who have a reasonable life
expectancy and favorable anatomy for transcatheter repair, percutaneous mitral valve repair is suggested.

Determining the need for intervention in patients with chronic primary MR is based upon the severity of MR, the
presence or absence of symptoms, LVESD and LVEF, the feasibility of valve repair, the presence of atrial fibrillation,
the presence of pulmonary hypertension at rest or with exercise, whether other cardiac surgery is required, and the
preference and expectations of the patient.
 Patients with chronic primary severe MR who develop symptoms (eg, dyspnea or fatigue) are candidates for
corrective mitral surgery, even if the symptoms improve with medical therapy or the LV appears to be compensated
If there is uncertainty about the presence or absence of symptoms, exercise testing may provide objective
information.

For symptomatic patients with chronic severe primary MR (stage D) with LVEF >30 percent, we recommend mitral
valve surgery. Surgery should be performed as soon as symptoms develop. For symptomatic patients with chronic
severe primary MR and LVEF 30 percent, with high likelihood of successful mitral repair, we suggest mitral valve
repair

For symptomatic patients with chronic severe primary MR and LVEF 30 percent, with mitral valve not amenable to
repair, we suggest not performing mitral valve surgery.

For asymptomatic patients Waiting for the patient to experience dyspnea or exercise intolerance may allow time
for the development of irreversible depression of LV function. For this reason, it is important to monitor LV size and
function in patients with asymptomatic MR. Some degree of LV and left atrial enlargement are to be expected in
severe chronic MR; thus, chamber enlargement alone is generally not an indication for surgery in patients with
severe chronic MR For asymptomatic patients with chronic severe primary MR and an LVEF of 30 to 60 percent
and/or an LVESD 40 mm (stage C2), we recommend mitral valve surgery. Patients with chronic severe primary MR
with an LVEF 30 percent would be expected to be symptomatic For asymptomatic patients with chronic severe or
moderate primary MR undergoing cardiac surgery for other indications, we recommend concomitant mitral valve
repair.

For asymptomatic patients with chronic severe nonrheumatic primary MR (stage C1) and preserved LV function
(LVEF >60 percent and LVESD <40 mm) in whom there is at least a 60 percent likelihood of a successful and durable
repair and who have new onset of atrial fibrillation or resting pulmonary hypertension (pulmonary artery systolic
arterial pressure >50 mmHg), we suggest surgical mitral valve repair.

Watchful waiting is appropriate for asymptomatic patients with chronic severe MR with normal LV systolic function
(with LVEF 60 percent and LVESD <40 mm) who lack criteria for intervention. The importance of surgical repair
before there is a significant impairment in LV function is well known

A separate issue is surgery in patients with severe chronic primary MR and severe LV dysfunction (LVEF <30 percent
and/or LVESD >55 mm) in whom surgery may improve symptoms but may not alter long-term mortality rates or need
for LV assist device support or transplantation. Such patients with primary mitral valve disease generally exhibit
irreversible depression of ventricular contractility due to long-standing volume overload. LV systolic function does not
improve after mitral valve surgery. For patients with severe MR with LVEF <30 percent who have a high likelihood of
durable repair and low comorbidity, some experts consider the potential benefit of mitral valve surgery with
attempted repair greater than the risk

Surgical mitral valve repair versus replacement For patients with chronic primary MR, when mitral valve repair is
feasible, it is generally preferred to mitral valve replacement based upon the following considerations .LV systolic
function is better preserved with mitral valve repair than with standard mitral valve replacement. However, mitral
valve replacement with chordal sparing preserves LV systolic function with results comparable to mitral valve repair.
However, the likelihood of successful and durable mitral valve repair depends upon mitral valve anatomy and surgical
expertise.

Transcatheter mitral valve repair For severely symptomatic patients (New York Heart Association class III to IV)
despite optimal guideline-directed medical therapy with chronic severe primary MR (stage D) who have a reasonable
life expectancy and favorable anatomy for transcatheter repair and have a prohibitive surgical risk because of severe
comorbidities, we suggest percutaneous mitral valve repair..)

MV surgery is recommended for symptomatic patients with chronic severe primary MR (stage D) and
LVEF >30%
MV surgery is recommended for asymptomatic patients with chronic severe primary MR and LV
dysfunction (LVEF 30 to 60% and/or LVESD 40 mm, stage C2)
MV repair is recommended in preference to MVR when surgical treatment is indicated for patients
with chronic severe primary MR limited to the posterior leaflet
MV repair is recommended in preference to MVR when surgical treatment is indicated for patients
with chronic severe primary MR involving the anterior leaflet or both leaflets when a successful and
durable repair can be accomplished
Concomitant MV repair or replacement is indicated in patients with chronic severe primary MR
undergoing cardiac surgery for other indications
MV repair is reasonable in asymptomatic patients with chronic severe primary MR (stage C1) with
preserved LV function (LVEF >60% and LVESD <40 mm) in whom the likelihood of a successful and
durable repair without residual MR is >95% with an expected mortality rate of <1% when performed at
a Heart Valve Center of Excellence
MV repair is reasonable for asymptomatic patients with chronic severe nonrheumatic primary MR
(stage C1) and preserved LV function in whom there is a high likelihood of a successful and durable
repair with 1) new onset of AF or 2) resting pulmonary hypertension (pulmonary artery systolic
IIa B
pressure >50 mmHg)
Concomitant MV repair is reasonable in patients with chronic moderate primary MR (stage B)
undergoing cardiac surgery for other indications
IIa C
MV surgery may be considered in symptomatic patients with chronic severe primary MR and LVEF
30% (stage D)
IIb C
MV repair may be considered in patients with rheumatic mitral valve disease when surgical treatment
is indicated if a durable and successful repair is likely or if the reliability of long-term anticoagulation IIb B
management is questionable
Transcatheter MV repair may be considered for severely symptomatic patients (NYHA class III/IV) with
chronic severe primary MR (stage D) who have a reasonable life expectancy but a prohibitive surgical IIb B
risk because of severe comorbidities
MVR should not be performed for treatment of isolated severe primary MR limited to less than one-
half of the posterior leaflet unless MV repair has been attempted and was unsuccessful

Valve replacemnt
bioprosthesis is recommended in patients of any age for whom anticoagulant therapy is contraindicated, cannot be
managed appropriately, or is not desired
A mechanical prosthesis is reasonable for AVR or MVR in patients <60 y of age who do not have a contraindication
to anticoagulation
A bioprosthesis is reasonable in patients >70 y of age
Either a bioprosthetic or mechanical valve is reasonable in patients between 60 y and 70 y of age
Replacement of the aortic valve by a pulmonary autograft (the Ross procedure), when performed by an
experienced surgeon, may be considered in young patients when VKA anticoagulation is contraindicated or
undesirable

Management of patients with chronic secondary mitral regurgitation

Secondary MR (also known as functional MR) is caused by left ventricular dysfunction

caused by coronary heart disease or by a cardiomyopathy.) Staging of secondary mitral
regurgitation (MR) is based upon symptoms, valve anatomy, and valve hemodynamics
(severity of MR), which are associated with left ventricular (LV) dysfunction (due to
coronary artery disease or cardiomyopathy)

In stage A, patients are at risk of MR (or have mild MR). Mitral valve leaflets, chords,
and annulus are normal with no or mild MR (small central jet <20 percent of left atrium
on color Doppler with vena contracta <0.3 cm). Patients with coronary artery disease
have normal or mildly dilated LV size with fixed (myocardial infarction) or inducible
(ischemia) regional wall motion abnormalities. Patients with cardiomyopathy have
abnormal LV geometry, usually with dilation and systolic dysfunction.

In stage B, patients have progressive (moderate) MR, defined as an effective

regurgitant orifice <0.20 cm2, regurgitant volume <30 mL, and regurgitant fraction <50
percent. Patients with coronary artery disease have regional wall motion abnormalities
and reduced LV systolic function. Patients with dilated cardiomyopathy exhibit annular
dilation with mild loss of central coaptation of the mitral leaflets and syst dysfunction.

In stage C, patients have asymptomatic severe MR, defined as an effective regurgitant

orifice 0.20 cm2, regurgitant volume 30 mL, and regurgitant fraction 50 percent.
Patients with coronary artery disease have regional wall motion abnormalities and/or
LV dilation with severe tethering of mitral leaflets and reduced LV systolic function.
Patients with cardiomyopathy have LV dilation with associated annular dilation with
severe loss of central coaptation of the mitral leaflets and systolic dysfunction. This
category includes patients with symptoms due to myocardial ischemia or heart failure
that respond favorably to revascularization and medical therapy.

In stage D, patients have symptomatic severe MR (as defined above). Symptoms may
include decreased exercise tolerance and exertional dyspnea. Heart failure symptoms
due to MR often persist even after revascularization and optimization of medical tx

Chronic secondary mitral regurgitation (MR) develops as a consequence of left

ventricular (LV) systolic dysfunction . Patients with chronic secondary MR and HFrEF
should receive standard therapy for HFrEF, including angiotensin converting enzyme
inhibitor (ACE) inhibitor (or angiotensin II receptor blocker [ARB], or angiotensin
receptor-neprilysin inhibitor [ARNI]), beta blocker and mineralocorticoid receptor
antagonist, and diuretic therapy as needed to treat volume overload.

Patients with chronic secondary MR should receive cardiac resynchronization therapy

according to standard guidelines

antibiotic prophylaxis is not recommended when patients with mitral valve disease (in
the absence of prosthetic repair or replacement or history of infective endocarditis)

Isolated mitral valve surgery (repair or replacement) for severe chronic secondary MR
is not generally recommended since there is no proven mortality benefit and uncertain
durable effect on symptoms. For patients with chronic severe secondary MR who are
undergoing coronary artery bypass graft surgery (CABG) or surgical aortic valve
replacement, we suggest mitral valve surgery. The available evidence suggests that for
patients with severe ischemic MR, survival is similar following mitral valve
replacement with chordal sparing and mitral valve repair. However, recurrent MR is
much more frequent following mitral valve repair. For patients with nonischemic MR
who undergo mitral valve surgery, we suggest mitral valve replacement with chordal
sparing unless valve anatomy is favorable for repair and intraoperative transesophageal
echocardiography demonstrates minimal residual MR after repair.

Transcatheter mitral valve repair Observational data suggest this device can also
reduce the severity of MR and improve functional class in patients with secondary MR

PROGNOSIS Secondary mitral regurgitation (MR) is associated with adverse prognosis in patients with ischemic
or nonischemic cardiomyopathy, although there is no evidence that surgical correction of secondary MR improves
survival. . Severe MR was an independent predictor of mortality in pt with EF < 35 %, others included severity of
tricuspid regurgitation . Right ventricular dysfunction may be a predictor of poor outcome among patients with
secondary MR. Ischemic MR following myocardial infarction (MI) is associated with increased mortality as well as
risk of development of HF . Ischemic MR is also an important predictor of the development of HF, even in patients
with a normal LVEF at the time of the MI.
Tricuspid regurgitation (TR)

A small degree of tricuspid regurgitation (TR) is present in approximately 70 percent of

normal adults. Abnormal degrees of TR in adults are largely functional in the setting of
right ventricular pressure and/or volume overload) and much less often due to primary
disorders of the valve

TR in adults is most commonly functional, defined as regurgitation with apparently

anatomically normal leaflets and chords. The cause of functional TR most likely is
dilatation of the right atrium and right ventricle with dilation of the tricuspid annulus
and may result from any condition that directly involves the right ventricle or causes
elevation in right ventricular systolic pressure often with pulmonary hypertension.

The disorders that induce pulmonary hypertension and secondary right ventricular
dilatation include the following: Left-sided heart failure. Mitral stenosis or
regurgitation. Primary pulmonary disease cor pulmonale, pulmonary embolism,
pulmonary hypertension of any cause.Left to right shunt atrial septal defect,
ventricular septal defect, anomalous pulmonary venous return. Eisenmenger
syndrome. Stenosis of the pulmonic valve or pulmonary artery. Hyperthyroidism.
Examples of right ventricular diseases causing functional TR include right ventricular
myocardial infarction and various types of cardiomyopathy affecting the right ventricle.

Causes of primary TR include:Direct valve injury from a permanent pacemaker or

implantable cardioverter-defibrillator lead placement or removal or from
endomyocardial biopsy in cardiac transplant recipientsChest trauma.Infective
endocarditis.Ebstein's anomalyRheumatic fever.Carcinoid syndrome. Ischemic
heart disease affecting the right ventricle with papillary muscle dysfunction or
rupture.Myxomatous degeneration associated with tricuspid valve prolapse, which
occurs in as many as 40 percent of patients with prolapse of the mitral valve.
(Connective tissue disorder (eg, Marfan syndrome).Marantic endocarditis in systemic
lupus erythematosus or rheumatoid arthritis.Drug-induced disease.

Tricuspid regurgitation (TR) is characterized by the backflow of blood into the right
atrium during systole. Since the right atrium is relatively compliant, there are often no
major hemodynamic consequences with mild or moderately severe TR. However, when
TR is severe, right atrial and venous pressure rise and can result in the signs and of
right-sided heart failure. In such patients, right ventricular pressure and/or volume
overload frequently lead to right ventricular systolic dysfunction and a low forward
cardiac output. Elevated central venous pressure in patients with severe TR may
contribute to impaired renal function regardless of the etiology of the regurgitant lesion.
Such an effect is not closely related to the cardiac output

The most prominent features of the physical examination in patients with tricuspid
regurgitation (TR) are those related to the regurgitant murmur and the development of
right-sided heart failure. With severe right heart failure, the patient often looks
cachectic, chronically ill, cyanotic, and occasionally jaundiced (reflecting hepatic
dysfunction). If TR is due to left ventricular dysfunction, signs of left-sided heart failure
may dominate.
Distended and prominent jugular veins ,Ascites and peripheral edema , anasarca
unilateral or bilateral pleural effusions .Hepatomegaly

TR is classically associated with a holosystolic murmur that is best heard at the right or
left mid sternal border or at the subxiphoid area. Interventions that result in an increase
in venous return (leg raising, exercise, hepatic compression) will augment the murmur
of TR. . On the other hand, reducing venous return (with standing or amyl nitrate) will
diminish the intensity of the murmur. Respiratory variation in the intensity and duration
of the murmur (Rivero-Carvallo's sign) may be observed with mild to moderate TR.
With inspiration, there is an increase in venous return to the right ventricle; as a result,
the murmur of TR becomes louder and may become longer

Electrocardiography and chest radiography may reveal findings reflective of right atrial
and right ventricular enlargement and concomitant left heart disease, if present.
Echocardiography is the main diagnostic modality . Cardiovascular magnetic resonance
may be helpful

tion, right ventricular heave, peripheral edema, ascites, and hepatomegaly. A

holosystolic murmur of TR may be heard but this may be soft or absent even when the
TR is severe. (See 'Physical examination' above.)

Electrocardiography and chest radiography may reveal findings reflective of right

atrial and right ventricular enlargement and concomitant left heart disease, if present.
(See 'Testing' above.)

Echocardiography is the main diagnostic modality for evaluation of TR. It enables

evaluation of the severity of TR, valve morphology, right chamber sizes and right
ventricular function, estimation of pulmonary artery systolic pressure as well as
assessment of any concomitant left heart disease. Cardiovascular magnetic resonance
may be helpful if further evaluation such as quantitation of tricuspid regurgitant
fraction, right ventricular volumes, and ejection fraction is indicated.

While the clinical setting (particularly concomitant cardiovascular disease) influences

survival severe TR is an independent predictor of mortality

In patients with severe tricuspid regurgitation (TR) and right-sided heart failure,
diuretics are suggested to treat volume overload,. Loop diuretics are typically used.
Aldosterone antagonists may provide additional benefit

Most adults with TR have significant left-sided heart disease and treatment should be
directed at the primary disease process. However, many patients with severe TR have
refractory edema unresponsive to medical management.

Reduction of high pulmonary artery pressures may help reduce functional TR (ie, due
to pulmonary hypertension without intrinsic tricuspid valvular disease)

Indications for tricuspid valve surgery depend upon whether surgery for left-sided
(mitral or aortic) valve disease is indicated For patients with severe tricuspid
regurgitation (TR) who are undergoing left-sided valve surgery, tricuspid valve surgery
is recommended, . Primary or functional severe TR does not consistently improve after
treatment of a left-sided valve lesion (with reduction of right ventricular afterload). For
patients with mild, moderate, or greater functional TR who are undergoing left-sided
valve surgery, concomitant tricuspid valve repair is suggested if there is either 1)
tricuspid annular dilation (diameter on transthoracic echocardiogram of >40 mm or 21
mm/m2 indexed for body surface area or intraoperative diameter>70 mm) or 2) prior
evidence of right heart failure.

Isolated tricuspid surgery - Tricuspid valve surgery is suggested (weak

recommendation) for patients with severe primary TR with symptoms unresponsive to
medical therapy, preferably before the onset of significant right ventricular dysfunction.
surgery should be considered in patients with severe isolated TR with mild or no
symptoms and progressive right ventricular dilation or deterioration of right ventricular
function

tricuspid valve repair is generally preferred to tricuspid valve replacement, with valve
replacement performed only when repair is not feasible . Tricuspid repair procedures
include placement of a prosthetic annular ring (eg, the Carpentier-Edwards ring and the
Cosgrove-Edwards band) or a biologic annuloplasty repair .Recurrence of TR is
common following tricuspid valve repair