Mitral valve prolapse

Highlights
Summary Overview

Basics
Definition Epidemiology Aetiology Pathophysiology Classification

Prevention
Screening

Diagnosis
History & examination Tests Differential Step-by-step Criteria Guidelines Case history

Treatment
Details Step-by-step Emerging Guidelines

Follow Up
Recommendations Complications Prognosis

Resources
References Images Patient leaflets Credits Email Print Feedback Share Add to Portfolio Bookmark Add notes

History & exam

Key factors
presence of risk factors mid-systolic click late-systolic murmur

Other diagnostic factors

palpitations History & exam details

Diagnostic tests
1st tests to order
echocardiogram

Tests to consider
Holter or event monitor

Diagnostic tests details

Treatment details
Acute
asymptomatic o symptomatic o urgent evaluation + confirmation of diagnosis lifestyle modifications beta blockers aspirin or warfarin with severe regurgitation mitral valve repair + postoperative aspirin or warfarin or supportive medical therapy Treatment details reassurance aspirin or warfarin with severe mitral regurgitation mitral valve repair + postoperative aspirin or warfarin

Summary
A common valvular heart condition. One or both leaflets of the mitral valve prolapse into the left atrium during systole. Diagnosis established by clinical examination and echocardiogram. Usually follows a benign course with a favourable prognosis. Uncommonly, serious complications occur such as infective endocarditis, severe mitral regurgitation, or sudden cardiac death. Patients at increased risk identifiable by clinical and echocardiographical features. Mitral valve repair is the preferred treatment option in patients with mitral valve prolapse and severe mitral regurgitation.

Definition
Mitral valve prolapse (MVP) is defined as systolic prolapse or billowing of one or both mitral valve leaflets into the left atrium. [1] [2] MVP with leaflet thickening defined as 5 mm or greater during diastasis, reflecting myxomatous degeneration, is referred to as classic prolapse and is a predictor of complications. [3] [4] The degree of associated mitral regurgitation ranges from none to severe. The mitral valve and annulus are saddle shaped. [5] [6] The echocardiographic definition of MVP is prolapse of leaflet(s) by 2 mm or greater above the level of the annulus during systole in the long-axis parasternal or apical views on 2-dimensional imaging.View image In other echocardiographical views, such as the apical 4-chamber view, the leaflets may appear to prolapse even though they remain below the level of the entire mitral valve. This can lead, as happened in older studies, to an incorrect diagnosis of MVP. [7] [8]

Epidemiology
MVP is the most common valvular heart disease in the US. [1] According to current diagnostic criteria, the prevalence in the US is estimated at 2.4% with equal male and female distribution.[1] [3] In other countries the prevalence rate ranges from 2.5% to 15%. [11] [12] The risk of complications is greater in men and in people over 45 years old. [1] [4] Older echocardiographical studies used referral-based clinical studies and less-specific criteria for definition. Selection bias led to false high-incidence figures of up to 38% in some population groups. [7]

Aetiology
The exact aetiology of MVP is unknown. A genetic link has been identified in familial variants of MVP, including chromosomal abnormalities such as MMVP1 (chromosome 16p), [13] MMVP2 (chromosome 11p15), [14] and MMVP3 (chromosome 13q31). [15] Primary MVP is associated with characteristic histological changes of myxomatous degeneration. These include proliferation of the spongiosa layer (middle layer) of leaflet tissue due to abnormal glycosaminoglycan production, [16] [17] [18] abnormal leaflet collagen structure, [16] [17] and abnormally weak and stretchy leaflet chordae. [19] MVP with characteristic myxomatous degeneration occurs more frequently in certain connective tissue diseases such as Marfan's syndrome, [20] EhlersDanlos syndrome, [21]osteogenesis imperfecta, and pseudoxanthoma

elasticum. [22] This suggests that primary MVP may be a related connective tissue disorder.

Pathophysiology
Classic (primary) MVP
In classic MVP, progressive myxomatous changes in leaflets result in mitral regurgitation that may worsen over time. MVP-related mitral regurgitation produces volume overload of the left ventricle (LV) and left atrium. Preload increases and the LV dilates in order to maintain a normal forward flow. However, the increase in afterload resulting from LV dilatation is offset by the fact that the ventricle is pumping much of its volume, including regurgitant volume, into a low-impedance circuit, the left atrium. Therefore, afterload may be variably reduced initially in mitral regurgitation and typically becomes elevated only in later stages of the disease as LV size increases further. Thus, ejection indices such as ejection fraction are not considered reliable measures of LV contractile function and remain in the normal range when contractility is already impaired. [23] Chordal elongation and leaflet prolapse often result in chordal rupture with prompt deterioration in mitral regurgitation due to flail leaflet and loss of coaptation.

Non-classic (secondary or functional) MVP

Secondary or functional MVP occurs when histologically normal valves prolapse. This occurs due to imbalance of geometric features that normally govern mitral valve mechanical function such as LV size, mitral annular dimensions, and mitral leaflet size. For example, in younger women who are volume depleted, a disproportionately small LV cavity dimension may result in prolapse. [24] Another example is the occurrence of MVP associated with secundum atrial septal defects. [25]

Mitral regurgitation
rupture. Mitral regurgitation occurs in MVP either due to progressive myxomatous degeneration or due to chordal rupture with resultant flail segment. Progression of mitral regurgitation can be abrupt when related to chordal

Classification
Histology-based classification Classic or primary MVP involves histological changes in the valve leaflet. Non-classic MVP, also termed secondary or functional MVP, is the prolapse of histologically normal valves. Classic (primary)
Defined as leaflet thickening of 5 mm or greater during diastasis, reflecting myxomatous degeneration. These changes are predictors of complications. [3] [4] Most often sporadic, but familial variants are recognised.

Familial variants are typically inherited in an autosomally dominant fashion with incomplete penetrance. [8] [9] May be associated with connective tissue disorders such as in Marfan's syndrome.

Non-classic (secondary or functional)

Histologically normal valves prolapse. Involves mismatch between ventricular size and mitral valve apparatus, associated with a variety of conditions, including atrial septal defect, anorexia, hypertrophic cardiomyopathy, volume depletion, or papillary muscle ischaemia.

Leaflet involvement Disease may be classified based on the number of leaflets involved. Unileaflet disease is more common than bileaflet MVP. In unileaflet MVP, the posterior leaflet is more commonly affected. MVP syndrome Previously, a constellation of findings was associated with MVP and termed MVP syndrome. These findings included atypical chest pain, anxiety, syncope, palpitations, exercise intolerance, dyspnoea, low BP, and abnormal ECG. However, these associations were incorrect and probably reflected poor study design and selection bias in older studies. In the unselected outpatient population studied prospectively in the Framingham Heart Study, the only association found with MVP was low body weight. [10]

Screening
Screening of first-degree relatives
Echocardiography is used to screen first-degree relatives of patients with MVP. However, there is no consensus on this issue. [1] [31]

History & examination

Key diagnostic factorshide all
presence of risk factors (common)

Key factors include connective tissue diseases (such as Marfan's syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum), positive FHx, and slim body type.

mid-systolic click (common)

The combination of click and murmur is specific but not sensitive for diagnosis. [27]

Changes in timing with dynamic manoeuvres help to differentiate MVP from other causes. With a decrease in preload (standing, Valsalva) or decrease in afterload (amyl nitrate administration), the click and murmur occur earlier in systole. Conversely, with an increase in left ventricular volume (squatting) or increase in afterload (handgrip) the click and murmur occur later.

late-systolic murmur (common)

The duration and intensity of murmur vary with loading conditions. The murmur may progress to holosystolic with progressive severity of mitral regurgitation. With a decrease in preload (standing, Valsalva) or decrease in afterload (amyl nitrate administration), the click and murmur occur earlier in systole. Conversely, with an increase in left ventricular volume

(squatting) or increase in afterload (handgrip) the click and murmur occur later. Other diagnostic factorshide all palpitations (common)

There is no increased incidence of ECG abnormalities. [26] [10] Continuous ambulatory Holter or event monitoring is indicated. [1]

Risk factorshide all

Strong connective tissue disorders

MVP with characteristic myxomatous degeneration occurs more frequently in certain connective tissue diseases such as Marfan's syndrome, [20] Ehlers-Danlos syndrome, [21] osteogenesis imperfecta, and pseudoxanthoma elasticum. [22]

Weak FHx

This suggests that primary MVP may be a related connective tissue disorder.

In general, MVP is a sporadic condition, but familial clustering is well recognised as autosomally dominant with variable penetrance. [8] [9]

The familial variant is associated with an increased incidence of sudden cardiac death. [1] slim body type

This is associated with an increased risk of MVP.

Diagnostic tests
1st tests to orderhide all
Test

echocardiogram Key test indicated to confirm diagnosis and assess high-risk features.View image Sensitivity and specificity are high when current diagnostic criteria are used. Also useful for routine follow-up studies or for change in clinical status.

Predictors of poor outcome include moderate to severe mitral regurgitation, left ventricular ejection fraction <50%

ventricular dilatation [4] and leaflet thickening >5 mm. [3] [29] [30] The effect of chronic mitral regurgitation on lef impaired. [23]

ventricular systolic function can be latent, and ejection fraction can remain in the normal range when contractility

Tests to considerhide all

Test

Holter or event monitor arrhythmias. Some patients with MVP experience palpitations in the absence of findings on event monitoring. Indications for electrophysiological testing are similar to those of the general population.

Patients with palpitations or syncope are evaluated with Holter or event ambulatory monitoring to rule out signific

Differential diagnosis
Condition Differentiating signs/symptoms Differentiating tests

Aortic valve disease

Aortic ejection clicks occur earlier in systole than in MVP, best heard to the right of the upper sternum. Click timing not affected by dynamic manoeuvres.

Echocardiogram is diagnostic for bicuspid aortic valve disease as opening motion of the aortic valve in this condition.

Pulmonic valve disease

Pulmonic ejection clicks occur earlier in systole than in MVP, best heard to the left of the upper sternum. Click

Echocardiogram is diagnostic for pulmonic valvular disease as ab Doppler findings associated with this valve are evident.

timing not affected by dynamic manoeuvres. Atrial myxoma

May have symptoms of weight loss, fever, malaise. Examination may reveal a diastolic tumour plop or diastolic murmur similar to mitral stenosis.

Heterogeneous masses, may be sessile, typically in left atrium. T evaluation may reveal leukocytosis.

echocardiogram may yield more detail than transthoracic echoca

Ischaemia-related mitral regurgitation (MR)

History reveals previous MI. Examination features are similar to MVP-related regurgitant murmurs. May be late systolic or holosystolic, which can mimic severe MVP-related MR. Absence of click.

ECG reveals previous infarction. Echocardiogram findings may d

and left ventricular akinesis or dyskinesis. The posterior mitral va

restricted motion as compared with the excess motion seen in MV

Rheumatic heart disease-related MR

History of group A streptococcal pharyngitis

ECG may show heart block. Echocardiogram is diagnostic with e as it opens commonly accompanies the regurgitation.

motion of both leaflets. A degree of stenosis of the mitral valve wi

followed by migratory large-joint arthritis, fever, rash, and subcutaneous nodules. Rheumatic heart disease typically follows 1-2 decades later. Examination features are similar to MVP-related regurgitant murmurs. Usually holosytolic, which can mimic severe MVP-related MR. Absence of click. Infective endocarditis affecting mitral valve

Fever, chills, sweats, weight loss, Osler nodes, subungual haemorrhages , Janeway lesions, Roth spots, petechiae.

Vegetations on echocardiography, typically on the atrial side of th

Transoesophageal echocardiogram may be more sensitive than t seen.

echocardiogram. Leukocytosis, anaemia, elevated ESR, microsco

Tricuspid valve prolapse

While tricuspid

Echocardiogram is diagnostic but tricuspid valve prolapse and MV

prolapse may also produce a mid-systolic click sound, it is best heard at the lower left sternal border, or occasionally on the lower right parasternal area. Timing between the S1 and a tricuspid click often increases after inspiration or other manoeuvres that increase right ventricular return.

whereas isolated tricuspid valve prolapse is rare.

Step-by-step diagnostic approach

Often patients with MVP are asymptomatic, and auscultatory findings prompt further evaluation. Expert physical examination alone is highly sensitive for MVP, but specificity is limited. Diagnosis is typically established by echocardiography. [1]

Initial clinical evaluation

The classic finding on cardiac auscultation is a mid-systolic click, followed by a medium- to high-pitched, late-systolic murmur of varying duration. The click is generated by the tensing of the mitral apparatus as the leaflets prolapse. The murmur is caused by mitral regurgitation (MR). The typical mid- to late-

systolic click and murmur of MVP is distinct from the holosystolic murmur of torn chordae tendineae. Dynamic manoeuvres can be used to alter the timing of the click during systole. With a decrease in preload (standing, Valsalva) or decrease in afterload (amyl nitrate administration), the click and murmur occur earlier in systole. Conversely, with an increase in left ventricular volume (squatting) or increase in afterload (handgrip) the click and murmur occur later.

Echocardiogram
When clinical examination suggests MVP, an echocardiogram is indicated. This establishes the diagnosis by detecting leaflet prolapse of 2 mm above level of the annulus during systole in long-axis parasternal or apical views on 2-dimensional imaging.View image It also assesses additional important features such as the presence of left-sided chamber enlargement, leaflet morphology, and the presence and severity of MR. [1] The effect of chronic MR on left ventricular systolic function can be latent, and ejection fraction can remain in the normal range when contractility is already impaired. [23]

Palpitations or syncope
The correlation between palpitations and MVP is complex and incompletely understood. Evaluation of palpitations or syncope should mirror the work-up in patients without MVP, including evaluation with Holter or ambulatory event monitoring to rule out significant arrhythmias. The indications for electrophysiological testing are similar to those for the general population.
Click to view diagnostic guideline references.

Diagnostic criteria

Echocardiography
Prolapse of leaflet 2 mm or greater above the level of the annulus during systole in the long-axis parasternal or apical views on 2-dimensional imaging. [5] [6] View image

Case history
A 45-year-old man presents for a routine physical examination as part of an insurance medical assessment. He is asymptomatic and has no family history of cardiac disease or sudden cardiac death. On examination, he is of slim build. BP is 115/65 mmHg, and heart rate 60 bpm and regular. On cardiac examination, apex beat is of normal character and non-displaced. On

auscultation, he has a mid-systolic click followed by a late-systolic highpitched murmur. On standing and with Valsalva manoeuvre, the click and murmur occur earlier in systole and the murmur is of increased intensity. On squatting, the click and murmur occur later in systole and the murmur is softer in intensity. There are no clinical signs of heart failure.

Treatment Options
Treatment Patient group asymptomatic line 1st Treatmenthide all

reassurance

Asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged.

Mitral regurgitation is common in MVP. Most patients are asymptomatic. No specific therapy is required for mild mitral regurgitation.

adjunct [?]

aspirin or warfarin

American College of Cardiology/American Heart Association guidelines recommend antiplatelet therapy with aspirin in patients with MVP and atrial fibrillation (in patients younger than 65 years, with no mitral regurgitation, HTN, or CHF) or MVP and ischaemic stroke with no evidence of mitral regurgitation, atrial fibrillation, left atrial thrombus, or abnormal mitral leaflet; or MVP and a history of TIA. [1] [37]

Treatment with warfarin to a target INR of 2-3 is recommended for patients with MVP and previous stroke with evidence of mitral regurgitation, atrial fibrillation, or left atrial thrombus; MVP and atrial fibrillation with increased risk of thromboembolism (in patients older than 65 years, with mitral regurgitation, HTN, or CHF); MVP and recurrent TIAs despite aspirin therapy; or MVP and previous stroke with evidence of leaflet thickening (5 mm) or redundancy.[37]

Treatment Patient group asymptomatic line 1st Treatmenthide all

reassurance

Asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged.

Mitral regurgitation is common in MVP. Most patients are asymptomatic. No specific therapy is required for mild mitral regurgitation. Primary Options aspirin : 75-300 mg orally once daily OR warfarin : 5-10 mg orally once daily for 2 days initially, adjust dose to achieve INR of 2 to 3

with severe mitral regurgitation

adjunct [?]

mitral valve repair + postoperative aspirin or warfarin

Severe mitral regurgitation (MR) may warrant early consideration for surgical intervention. Surgery is indicated for left ventricular (LV) dysfunction (ejection fraction <60%, LV end-systolic diameter >40 mm). [1] Intervention is also considered for those with pulmonary HTN or atrial fibrillation. [1] [39]

There is some controversy over the management of patients without evidence of LV dysfunction, pulmonary HTN, or atrial fibrillation. Studies have reported increased cardiovascular mortality in this group despite the absence of symptoms. [40] American College of Cardiology/American Heart Association guidelines have recommended mitral valve surgery as reasonable for this group, in experienced surgical centres, and in cases where the likelihood of repair is >90%. [1] The European Society of Cardiology guidelines give this a lower level of

Treatment Patient group asymptomatic line 1st Treatmenthide all

reassurance

Asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged.

Mitral regurgitation is common in MVP. Most patients are asymptomatic. No specific therapy is required for mild mitral regurgitation. recommendation. [39]

Mitral valve repair is the preferred treatment option in patients with MVP and severe MR requiring surgery. These patients are usually treated in the postoperative period with aspirin and require anticoagulation only if atrial fibrillation is present. Percutaneous mitral valve repair is under investigation and is not yet FDA approved. Primary Options aspirin : 75 mg orally once daily Secondary Options warfarin : 5-10 mg orally once daily for 2 days initially, adjust dose to achieve INR of 2 to 3 More

symptomatic

1st

urgent evaluation + confirmation of diagnosis

Symptoms include palpitations, syncope, atypical chest pain, and anxiety. Evaluation of palpitations or syncope should mirror the work-up in patients without MVP, including evaluation with Holter or ambulatory event monitoring to rule out significant arrhythmias.

plus [?]

lifestyle modifications beta blockers

If Holter or event monitoring has been unrevealing,

Treatment Patient group asymptomatic line 1st Treatmenthide all

reassurance

Asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged.

Mitral regurgitation is common in MVP. Most patients are asymptomatic. No specific therapy is required for mild mitral regurgitation. a trial of lifestyle changes, including avoidance of stimulants, may be effective, especially in those with increased adrenergic responses to stress and activity. Avoidance of caffeine, nicotine and other stimulants may be beneficial. Reducing or eliminating alcohol may also be helpful. Regular exercise programme participation is generally advisable.

Restriction in competitive sport is recommended in the presence of moderate left ventricular enlargement or dysfunction, uncontrolled tachyarrhythmias, long QT interval, unexplained syncope, prior resuscitation from cardiac arrest, or aortic root enlargement. [41] [42]

Empirical beta-blocker treatment may be useful in alleviating symptoms of palpitations, anxiety, or atypical chest pain in certain patients. [36]

adjunct [?]

aspirin or warfarin

American College of Cardiology/American Heart Association guidelines recommend antiplatelet therapy with aspirin in patients with MVP and atrial fibrillation (in patients younger than 65 years, with no mitral regurgitation, HTN, or CHF) or MVP and ischaemic stroke with no evidence of mitral regurgitation, atrial fibrillation, left atrial thrombus, or abnormal mitral leaflet; or MVP and a history of TIA. [1] [37]

Treatment Patient group asymptomatic line 1st Treatmenthide all

reassurance

Asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged.

Mitral regurgitation is common in MVP. Most patients are asymptomatic. No specific therapy is required for mild mitral regurgitation.

Treatment with warfarin to a target INR of 2 to 3 is recommended for patients with MVP and previous stroke with evidence of mitral regurgitation, atrial fibrillation, or left atrial thrombus; MVP and atrial fibrillation with increased risk of thromboembolism (in patients older than 65 years, with mitral regurgitation, HTN, or CHF); MVP and recurrent TIAs despite aspirin therapy; or MVP and previous stroke with evidence of leaflet thickening (5 mm) or redundancy.[37] Primary Options aspirin : 75-300 mg orally once daily OR warfarin : 5-10 mg orally once daily for 2 days initially, adjust dose to achieve INR of 2 to 3

with severe regurgitation

plus [?]

mitral valve repair + postoperative aspirin or warfarin or supportive medical therapy

Both the American College of Cardiology/American Heart Association and the European Society of Cardiology guidelines stress the importance of performing mitral valve repair if feasible and referring patients to centres with appropriate surgical expertise. [1] [39] Mitral valve repair is technically possible in most instances in prolapse or flail of the posterior leaflet but is more difficult in anterior leaflet or flail, bileaflet prolapse. The

Treatment Patient group asymptomatic line 1st Treatmenthide all

reassurance

Asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged.

Mitral regurgitation is common in MVP. Most patients are asymptomatic. No specific therapy is required for mild mitral regurgitation. advantages of repair over replacement include lower operative and long-term mortality, better preservation of left ventricular systolic function, and avoidance of the need for anticoagulation associated with a prosthetic valve. Patients undergoing mitral valve repair are usually treated in the postoperative period with aspirin and require anticoagulation only if atrial fibrillation is present.

Non-surgical candidates with severe MR may warrant medical therapy for symptoms associated with inadequate cardiac output. Management typically includes agents to increase forward flow and improve contractility, including digoxin, vasodilators, angiotensin blockers, and diuretics. Primary Options aspirin : 75 mg orally once daily Secondary Options warfarin : 5-10 mg orally once daily for 2 days initially, adjust dose to achieve INR of 2 to 3 More

Acute

Treatment approach
Patients without symptoms generally require no therapy. MVP patients at high risk of embolism warrant consideration of anticoagulation. Antibiotic

prophylaxis before oral, GI, or GU procedures to prevent endocarditis is not recommended unless there is a prior history of endocarditis. [32] Increased risk of developing severe mitral regurgitation (MR) is associated with male sex, [33] age greater than 45 years, [34] elevated BMI, HTN, [35] left ventricular enlargement, left ventricular dysfunction, and thickened mitral valve leaflets. [3] [4] MVP-related MR is typically managed in a similar way to other forms of MR; however, surgical intervention may be more attractive for those with severe MR related to MVP.

Asymptomatic patients
Asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged.

Palpitations, anxiety, atypical chest pain

Palpitations and atypical symptoms warrant investigation similar to that in non-MVP patients. If the evaluation has been unrevealing, a trial of lifestyle changes, including avoidance of stimulants and alcohol, may be effective, especially in those with increased adrenergic responses to activity, stress, or stimulants. Empirical beta-blocker treatment may be useful in alleviating symptoms of palpitations, anxiety, or atypical chest pain in certain patients. [36]

Embolism risk
Patients with a history of TIA, stroke, or atrial fibrillation and MVP warrant consideration for anticoagulation. Aspirin or warfarin, depending on the severity of the risk of embolism, is recommended. [1] [37] [38] For asymptomatic MVP patients with echocardiographic findings suggesting a high risk for complications, available data do not support use of prophylactic aspirin unless patients meet the preceding criteria.

Mitral regurgitation
Mild MR
MR is common in MVP. Most patients are asymptomatic, and no specific therapy is required. Atrial fibrillation or indicators of haemodynamic compromise, such as left ventricular (LV) dysfunction or pulmonary HTN, are more typically associated with severe MR in MVP patients.

Severe MR

Patients with severe MR may warrant early consideration for surgical intervention, even when asymptomatic. Surgery is indicated for LV dysfunction (ejection fraction <60%, LV end-systolic diameter >40 mm). [1] Intervention for asymptomatic patients with severe MR is also considered for those with pulmonary HTN or atrial fibrillation. [1] [39]

There is some controversy over the management of patients with MVP and severe MR who are asymptomatic without evidence of LV dysfunction, pulmonary HTN, or atrial fibrillation. Studies have reported increased cardiovascular mortality in this group despite the absence of symptoms. [40] American College of Cardiology/American Heart Association guidelines have recommended mitral valve surgery as reasonable for this group, in experienced surgical centres, and in cases where the likelihood of repair is greater than 90%. [1] The European Society of Cardiology guidelines give this a lower level of recommendation. [39]

Mitral valve repair is the preferred treatment option in patients with MVP and severe MR requiring surgery. These patients are usually treated in the postoperative period with aspirin and require anticoagulation only if atrial fibrillation is present. Percutaneous mitral valve repair is under investigation.

Non-surgical candidates with severe MR may warrant medical therapy for symptoms associated with inadequate cardiac output. Management typically includes agents to increase forward flow and improve contractility, including digoxin, vasodilators, angiotensin blockers, and diuretics.

Emerging treatments
Percutaneous mitral valve repair Percutaneous mitral valve repair is a catheter-based treatment in which coaptation of the mitral leaflets is ameliorated by technologies deployed at the time of cardiac catheterisation. Two experimental approaches are under investigation. In one, a clip is used to approximate the centre of the mitral valve leaflets (e-Valve), thus giving a double orifice valve. In the other, a flexible ring is deployed and tightened in the coronary sinus so as to reduce the mitral annulus area. Initial studies (EVEREST I) of the mitral valve clip suggest that it reduces mitral regurgitation (MR) considerably in appropriately selected patients. [43] In follow-up of successfully treated patients for 12 months, 66% (50 of 76) survived without worsening MR or the need for mitral valve surgery. [44] A small trial (EVEREST II) is underway of the mitral valve clip versus surgical mitral valve repair. This technique appears to be best suited to a central jet of MR (where prolapse affects the central portion of either or both leaflets). Initial experience with mitral valve repair through the coronary sinus approach has been reported in humans. [45]

Monitoring
Asymptomatic patients without MR, or patients with mild regurgitation, are evaluated every 3 to 5 years. Echocardiography should be repeated in this group if symptoms develop or if clinical examination changes. [1] [39]

Patients with moderate MR should be followed up annually with repeat echocardiography. Patients with severe MR should be reviewed at least every 6 months with echocardiography. Stress echocardiography can be used in selected patients. There is no contraindication to pregnancy with MVP alone. [1]

Patient Instructions
Restriction in competitive sport is recommended in the presence of moderate left ventricular enlargement or dysfunction, uncontrolled tachyarrhythmias, long QT interval, unexplained syncope, prior resuscitation from cardiac arrest, or aortic root enlargement. [41] [42] Patients with increased adrenergic response to stress or activity demonstrated on event monitoring may benefit from lifestyle changes such as reducing alcohol, reducing stimulants such as coffee and colas, and engaging in a consistent exercise programme.

Complications
Complicationhide all

mitral regurgitation see our comprehensive coverage of Mitral regurgitation Patients at increased risk can be identified by clinical and echocardiographic features and require vigilant follow-up. [23] Increased risk of developing severe mitral regurgitation (MR) is associated with male sex, [33] age greater than 45 years, [34] elevated BMI, HTN, [35] left ventricular enlargement, left ventricular dysfunction, and thickened mitral valve leaflets. [3] [4] Only a small number of patients develop severe mitral regurgitation. Most patients are asymptomatic, and no specific therapy is required. Atrial fibrillation or indicators of haemodynamic compromise, such as left ventricular (LV) dysfunction or pulmonary HTN, are more typically associated with severe MR in MVP patients. Patients with severe MR may warrant early consideration for surgical intervention, even when asymptomatic. Surgery is indicated for LV dysfunction (ejection fraction <60%, LV end-systolic diameter >40 mm). [1] Intervention for asymptomatic patients with severe MR is also considered for those with pulmonary HTN or atrial fibrillation. [1][39] Mitral valve repair is the preferred treatment option. Nonsurgical candidates may warrant medical therapy for symptoms associated with inadequate cardiac output. Management typically includes agents to increase forward flow and improve contractility, including digoxin, vasodilators, angiotensin blockers, and diuretics. infective endocarditis see our comprehensive coverage of Infective endocarditis MVP is considered the most common cardiovascular condition predisposing to infective endocarditis, even though the absolute incidence of this complication in the MVP population is extremely low (estimated at 0.02%

per year).[46] Factors associated with increased risk include male sex, age over 45 years, [47] presence of MR, [28] and leaflet thickening greater than 5 mm. [3] [29] Morbidity and mortality are significant with a 5-year incidence of death or mitral valve surgery estimated to be 60%.[48] Routine antibiotic prophylaxis before procedures is not required, as the risk of endocarditis is low and there are few data to show that antibiotic prophylaxis reduces the risk of subsequent endocarditis. [32] Situations where prophylaxis may be indicated include those where endocarditis may pose a greater than normal risk such as prior history of endocarditis. Treatment of infective endocarditis generally involves a prolonged course of tailored antimicrobial therapy and in certain cases surgical intervention. sudden cardiac death see our comprehensive coverage of Cardiac arrest This is a rare complication of MVP, occurring in less than 2% of patients during long-term follow-up [4] with an estimated yearly rate of 0.004%. [49] The aetiology is uncertain but is thought to be related to ventricular arrhythmia. Factors associated with increased risk include the familial form of MVP, severe MR, and impaired left ventricular systolic function. [4] [49] Asymptomatic patients with evidence of severe MR should be vigorously followed up. Holter or event monitor should be done for symptoms of palpitations. Indications for electrophysiological testing are similar to those of the general population, including aborted sudden cardiac death, recurrent syncope of unknown cause, or symptomatic or sustained ventricular tachycardia. stroke see our comprehensive coverage of Overview of stroke There is disagreement as to whether MVP can cause strokes. The proposed mechanism is embolism formation due to release of platelet-fibrin thrombi from the leaflet surface. Patients with MVP who have strokes tend to have risk factors such as older age (>50 years), severe MR, atrial fibrillation, and thickened valve leaflets. [4] [50] Data suggest a yearly event rate of 0.7%. [51] MVP should rarely be considered the sole source of stroke. Anticoagulation is used in appropriate patients.

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Prognosis
Most patients with MVP have an excellent prognosis similar to age- and sexmatched controls.[4] A small number will develop serious complications that include infective endocarditis, severe mitral regurgitation (MR), and sudden cardiac death.

Patients at increased risk can be identified by clinical and echocardiographic features and will require vigilant follow-up. [23] Increased risk of developing severe MR is associated with male sex, [33] age greater than 45 years, [34] elevated BMI, HTN, [35] left ventricular enlargement, left ventricular dysfunction, and thickened mitral valve leaflets. [3] [4]