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Mitral valve prolapse

From Wikipedia, the free encyclopedia

Mitral valve prolapse (MVP; a.k.a. floppy mitral valve


syndrome, systolic click murmur syndrome or billowing Mitral valve pr olapse
mitral leaflet) is a valvular heart disease characterized by
the displacement of an abnormally thickened mitral valve
leaflet into the left atrium during systole.[1] It is the primary
form of myxomatous degeneration of the valve. There are
various types of MVP, broadly classified as classic and
nonclassic. In its nonclassic form, MVP carries a low risk of
complications and often can be kept minimal by dietary
attention. In severe cases of classic MVP, complications
include mitral regurgitation, infective endocarditis,
congestive heart failure, and, in rare circumstances, cardiac
arrest.
In mitral valve prolapse, the leaflets of the mitral
The diagnosis of MVP depends upon echocardiography,
which uses ultrasound to visualize the mitral valve. The valve prolapse back into the left atrium.
prevalence of MVP is estimated at 2–3% of the Classification and external resources
population.[1] Specialty Cardiology
The condition was first described by John Brereton Barlow ICD-10 I34.1
in 1966. In consequence, it may also be referred to as ICD-9-CM 394.0, 424.0
Barlow's syndrome,[2] and was subsequently termed mitral
OMIM 157700
valve prolapse by J. Michael Criley.[3]
DiseasesDB 8303
MedlinePlus 000180
Contents eMedicine emerg/316
Patient UK Mitral valve prolapse
1 Signs and symptoms
1.1 Murmur MeSH D008945
1.2 Mitral valve prolapse syndrome
1.3 Mitral regurgitation
2 Risk factors
3 Mechanism
4 Diagnosis
4.1 Classic versus nonclassic
4.2 Symmetric versus asymmetric
4.3 Flail versus non-flail
5 Prognosis
6 Treatment
6.1 Prevention of infective endocarditis
7 Epidemiology
8 History
9 References
10 Further reading
11 External links

Signs and symptoms


Murmur
Upon auscultation of an individual with mitral valve prolapse, a mid-systolic click, followed by a late systolic
murmur heard best at the apex is common. The length of the murmur signifies the time period over which
blood is leaking back into the left atrium, known as regurgitation. A murmur that lasts throughout the whole of
systole is known as a holo-systolic murmur. A murmur that is mid to late systolic, although typically associated
with less regurgitation, can still be associated with significant hemodynamic consequences.[4]

In contrast to most other heart murmurs, the murmur of mitral valve prolapse is accentuated by standing and
valsalva maneuver (earlier systolic click and longer murmur) and diminished with squatting (later systolic click
and shorter murmur). The only other heart murmur that follows this pattern is the murmur of hypertrophic
cardiomyopathy. A MVP murmur can be distinguished from a hypertrophic cardiomyopathy murmur by the
presence of a mid-systolic click which is virtually diagnostic of MVP. The handgrip maneuver diminishes the
murmur of an MVP and the murmur of hypertrophic cardiomyopathy. The handgrip maneuver also diminishes
the duration of the murmur and delays the timing of the mid-systolic click.[5]

Both valsalva maneuver and standing decrease venous return to the heart thereby decreasing left ventricular
diastolic filling (preload) and causing more laxity on the chordae tendineae. This allows the mitral valve to
prolapse earlier in systole, leading to an earlier systolic click (i.e. closer to S1), and a longer murmur.

Mitral valve prolapse syndrome

Historically, the term mitral valve prolapse syndrome has been applied to MVP associated with palpitations,
atypical chest pain, dyspnea on exertion, low body mass index, and electrocardiogram abnormalities in the
setting of anxiety, syncope, low blood pressure, and other signs suggestive of autonomic nervous system
dysfunction.[1]

Occasionally, supraventricular arrhythmias observed in MVP are associated with increased parasympathetic
tone.[6]

Mitral regurgitation

Mitral valve prolapse is frequently associated with mild mitral


regurgitation,[7] where blood aberrantly flows from the left ventricle into
the left atrium during systole. In the United States, MVP is the most
common cause of severe, non-ischemic mitral regurgitation.[1] This is
occasionally due to rupture of the chordae tendineae that support the mitral
valve.[5]

Risk factors
MVP may occur with greater frequency in individuals with Ehlers-Danlos
syndrome, Marfan syndrome[8] or polycystic kidney disease.[9] Other risk
factors include Graves disease[10] and chest wall deformities such as
pectus excavatum.[11] For unknown reasons, MVP patients tend to have a
low body mass index (BMI) and are typically leaner than individuals
without MVP.[12][13]

Rheumatic fever is common worldwide and responsible for many cases of


damaged heart valves. Chronic rheumatic heart disease is characterized by Mitral valve prolapse can result in
repeated inflammation with fibrinous resolution. The cardinal anatomic mitral regurgitation, shown here, in
which blood abnormally flows from
changes of the valve include leaflet thickening, commissural fusion, and
the left ventricle back into the left
shortening and thickening of the tendinous cords.[14] The recurrence of
atrium.
rheumatic fever is relatively common in the absence of maintenance of low
dose antibiotics, especially during the first three to five years after the first
episode. Heart complications may be long-term and severe, particularly if
valves are involved. Rheumatic fever, since the advent of routine penicillin administration for Strep throat, has
become less common in developed countries. In the older generation and in much of the less-developed world,
valvular disease (including mitral valve prolapse, reinfection in the form of valvular endocarditis, and valve
rupture) from undertreated rheumatic fever continues to be a problem.[15]

In an Indian hospital between 2004 and 2005, 4 of 24 endocarditis patients failed to demonstrate classic
vegetations. All had rheumatic heart disease (RHD) and presented with prolonged fever. All had severe
eccentric mitral regurgitation (MR). (One had severe aortic regurgitation (AR) also.) One had flail posterior
mitral leaflet (PML).[16]

Mechanism
The mitral valve, so named because of its resemblance to a bishop's mitre, is the heart
valve that prevents the backflow of blood from the left ventricle into the left atrium of the
heart. It is composed of two leaflets, one anterior and one posterior, that close when the
left ventricle contracts.

Each leaflet is composed of three layers of tissue: the atrialis, fibrosa, and spongiosa.
Patients with classic mitral valve prolapse have excess connective tissue that thickens the
spongiosa and separates collagen bundles in the fibrosa. This is due to an excess of St. Zenon of
dermatan sulfate, a glycosaminoglycan. This weakens the leaflets and adjacent tissue, Verona wearing a
resulting in increased leaflet area and elongation of the chordae tendineae. Elongation of mitre.
the chordae tendineae often causes rupture, commonly to the chordae attached to the
posterior leaflet. Advanced lesions—also commonly involving the posterior leaflet—lead
to leaflet folding, inversion, and displacement toward the left atrium.[12]

Diagnosis
Echocardiography is the most useful
method of diagnosing a prolapsed mitral
valve. Two- and three-dimensional
echocardiography are particularly valuable
as they allow visualization of the mitral
leaflets relative to the mitral annulus. This
allows measurement of the leaflet thickness
and their displacement relative to the Transesophageal echocardiogram of mitral valve prolapse.
annulus. Thickening of the mitral leaflets
>5 mm and leaflet displacement >2 mm
indicates classic mitral valve prolapse.[12]

Prolapsed mitral valves are classified into several subtypes, based on leaflet thickness, type of connection to the
mitral annulus, and concavity. Subtypes can be described as classic, nonclassic, symmetric, asymmetric, flail,
or non-flail.[12]

All measurements below refer to adult patients; applying them to children may be misleading.

Classic versus nonclassic

Prolapse occurs when the mitral valve leaflets are displaced more than 2 mm above the mitral annulus high
points. The condition can be further divided into classic and nonclassic subtypes based on the thickness of the
mitral valve leaflets: up to 5 mm is considered nonclassic, while anything beyond 5 mm is considered classic
MVP.[12]

Symmetric versus asymmetric


Classical prolapse may be subdivided into
symmetric and asymmetric, referring to the
point at which leaflet tips join the mitral
annulus. In symmetric coaptation, leaflet tips
meet at a common point on the annulus.
Asymmetric coaptation is marked by one
leaflet displaced toward the atrium with respect
to the other. Patients with asymmetric prolapse
are susceptible to severe deterioration of the
mitral valve, with the possible rupture of the
chordae tendineae and the development of a
flail leaflet.[12]
Diagnosis of mitral valve prolapse is based on modern
Flail versus non-flail echocardiographic techniques which can pinpoint abnormal leaflet
thickening and other related pathology.
Asymmetric prolapse is further subdivided into
flail and non-flail. Flail prolapse occurs when a
leaflet tip turns outward, becoming concave toward the left atrium, causing the deterioration of the mitral valve.
The severity of flail leaflet varies, ranging from tip eversion to chordal rupture. Dissociation of leaflet and
chordae tendineae provides for unrestricted motion of the leaflet (hence "flail leaflet"). Thus patients with flail
leaflets have a higher prevalence of mitral regurgitation than those with the non-flail subtype.[12]

Prognosis
Generally, MVP is benign. However, MVP patients with a murmur, not just an isolated click, have an increased
mortality rate of 15-20%.[17] The major predictors of mortality are the severity of mitral regurgitation and the
ejection fraction.[18]

Treatment
Individuals with mitral valve prolapse, particularly those without symptoms, often require no treatment.[19]
Those with mitral valve prolapse and symptoms of dysautonomia (palpitations, chest pain) may benefit from
beta-blockers (e.g., propranolol). Patients with prior stroke and/or atrial fibrillation may require blood thinners,
such as aspirin or warfarin. In rare instances when mitral valve prolapse is associated with severe mitral
regurgitation, mitral valve repair or surgical replacement may be necessary. Mitral valve repair is generally
considered preferable to replacement. Current ACC/AHA guidelines promote repair of mitral valve in patients
before symptoms of heart failure develop. Symptomatic patients, those with evidence of diminished left
ventricular function, or those with left ventricular dilatation need urgent attention.

Prevention of infective endocard itis

Individuals with MVP are at higher risk of bacterial infection of the heart, called infective endocarditis. This
risk is approximately three- to eightfold the risk of infective endocarditis in the general population.[1] Until
2007, the American Heart Association recommended prescribing antibiotics before invasive procedures,
including those in dental surgery. Thereafter, they concluded that "prophylaxis for dental procedures should be
recommended only for patients with underlying cardiac conditions associated with the highest risk of adverse
outcome from infective endocarditis."[20]

Many organisms responsible for endocarditis are slow-growing and may not be easily identified on routine
blood cultures (these fastidious organisms require special culture media to grow). These include the HACEK
organisms, which are part of the normal oropharyngeal flora and are responsible for perhaps 5 to 10% of
infective endocarditis affecting native valves. It is important when considering endocarditis to keep these
organisms in mind.
Epidemiology
Prior to the strict criteria for the diagnosis of mitral valve prolapse, as described above, the incidence of mitral
valve prolapse in the general population varied greatly.[12] Some studies estimated the incidence of mitral valve
prolapse at 5 to 15 percent or even higher.[21] One study suggested MVP in up to 35% of healthy teenagers.[22]

Recent elucidation of mitral valve anatomy and the development of three-dimensional echocardiography have
resulted in improved diagnostic criteria, and the true prevalence of MVP based on these criteria is estimated at
2-3%.[1] As part of the Framingham Heart Study, for example, the prevalence of mitral valve prolapse in
Framingham, MA was estimated at 2.4%. There was a near-even split between classic and nonclassic MVP,
with no significant age or sex discrimination.[13] MVP is observed in 7% of autopsies in the United States.[17]

History
The term mitral valve prolapse was coined by J. Michael Criley in 1966 and gained acceptance over the other
descriptor of "billowing" of the mitral valve, as described by John Brereton Barlow.[23]

References
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tps://www.ncbi.nlm.nih.gov/pubmed/4159172). doi:10.1016/0002-8703(66)90179-7 (https://doi.org/10.1
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3. Criley JM, Lewis KB, Humphries JO, Ross RS (July 1966). "Prolapse of the mitral valve: clinical and
cine-angiocardiographic findings" (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC459076). Br Heart J.
28 (4): 488–96. PMC 459076 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC459076) .
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Donald; O’Rourke, Robert A.; Dell’Italia, Louis J. (August 2009). "Mitral Valve Prolapse With a Late-
Systolic Regurgitant Murmur May Be Associated With Significant Hemodynamic Consequences". The
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3e31819d5ec6).
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(5): 309–17. PMID 3053383 (https://www.ncbi.nlm.nih.gov/pubmed/3053383).
8. "Related Disorders: Mitral Valve Prolapse" (https://web.archive.org/web/20070225111027/http://www.m
arfan.org/nmf/GetContentRequestHandler.do?menu_item_id=80). Archived from the original (http://ww
w.marfan.org/nmf/GetContentRequestHandler.do?menu_item_id=80) on 2007-02-25. Retrieved
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9. Lumiaho, A; Ikäheimo, R; Miettinen, R; Niemitukia, L; Laitinen, T; Rantala, A; Lampainen, E; Laakso,
M; Hartikainen, J (2001). "Mitral valve prolapse and mitral regurgitation are common in patients with
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PMID 11728952 (https://www.ncbi.nlm.nih.gov/pubmed/11728952). doi:10.1053/ajkd.2001.29216 (http
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(5): 1281–90. PMID 3554946 (https://www.ncbi.nlm.nih.gov/pubmed/3554946). doi:10.1016/0002-
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(https://doi.org/10.1016%2FS0735-1097%2885%2980021-8).
23. Barlow JB, Bosman CK (1966). "Aneurysmal protrusion of the posterior leaflet of the mitral valve. An
auscultatory-electrocardiographic syndrome". Am Heart J. 71 (2): 166–78. PMID 4159172 (https://www.
ncbi.nlm.nih.gov/pubmed/4159172). doi:10.1016/0002-8703(66)90179-7 (https://doi.org/10.1016%2F00
02-8703%2866%2990179-7).

Further reading
Confronting Mitral Valve Prolapse Syndrome by Lyn Frederickson, 1992, ISBN 0-44639-407-6
Taking Control: Living With the Mitral Valve Prolapse Syndrome by Kristine A. Scordo, 2006, ISBN 1-
42431-576-X
Mitral Valve Prolapse: A Comprehensive Patient's Guide to a Happier and Healthier Life by Ariel
Soffer, M.D., 2007, ISBN 0-61515-205-8
Natural Therapies for Mitral Valve Prolapse - How Diet and Nutritional Supplements Can Ease The
Symptoms of this Common Disorder, by Dr. Ronald Hoffman

External links
Mitral valve prolapse at DMOZ

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