Mitral Valve Prolapse


Article Author:
Sandy Shah


Article Editor:
Tony Oliver


Editors In Chief:
Sebastiano Cassaro
Joseph Lee
Tanya Egodage


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
1/23/2019 12:00:06 PM

Introduction

Mitral valve prolapse (MVP), also known also known as floppy mitral valve syndrome, systolic click-murmur syndrome, and billowing mitral leaflets, is a valvular heart disease disorder. It is a benign condition. In rare cases, it may present with sudden cardiac death, endocarditis or a stroke.[1][2][3]

MVP is usually identified during a clinical exam. Echocardiography confirms the diagnosis. This disorder is the most common cause of Non-ischemic mitral valve regurgitation in developed countries. [4]Symptomatic patients may need Mitral valve repair.

Etiology

MVP usually occurs as an isolated condition in connective tissue disorders such as Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, and aneurysms-osteoarthritis syndrome.[5]

Epidemiology

MVP is a common valvular disorder that affects 2% to 3% of the general population. This disorder affects approximately seven to eight million individuals in the United States and more than 16 million people worldwide. MVP is seen more frequently in individuals with Ehler-Danlos Syndrome, Marfan Syndrome, Polycystic kidney disease, Graves' disease, and pectus excavatum. Individuals with MVP tend to have a low body mass index and are typically leaner individuals than individuals without MVP. MVP can be associated with significant mitral valve regurgitation (4%), bacterial endocarditis, congestive heart failure, and even sudden death.[6]

Most MVP individuals have mitral valve regurgitation either mild, trace, or none.  

Pathophysiology

MVP is primary myxomatous degeneration of one or both leaflets of the mitral valve. Endothelium disruption leads to complications such as infectious endocarditis and thromboembolism. Most MVP individuals have minimal mitral valve structure derangement which is not clinically significant. [7]

Histopathology

Histologically, MVP is characterized as a myxomatous lesion. The spongiosa of the mitral valve leaflets proliferates with mucopolysaccharide deposits and excessive water content. Thus resulting in leaflet thickening and redundancy. Type III collagen content increases and elastin fibers are fragmented. 

History and Physical

MVP is associated with symptoms of atypical chest pain, palpitations, dyspnea on exertion, and exercise intolerance. Other symptoms such as anxiety, low blood pressure, and syncope suggest autonomic nervous system dysfunction. Occasionally, supraventricular arrhythmias are seen suggesting increased parasympathetic tone.

In MVP, the mid-systolic click is followed by last systolic murmur. This finding is commonly heard at the apex. The murmur of MVP varies with position. The murmur is accentuated when the patient is standing and in Valsalva maneuver (systolic click comes earlier, and murmur is longer) and diminishes when the patient is squatting (systolic click comes later, and murmur is shorter).

The murmur of MVP is similar to the murmur of hypertrophic cardiomyopathy. A mid-systolic click is diagnostic of MVP. The handgrip maneuver increases the murmur of MVP and decreases murmur of hypertrophic cardiomyopathy. The handgrip maneuver also decreases the duration of the murmur and delays the timing of the mid-systolic click in MVP.

Evaluation

The most useful method of making a diagnosis of MVP is by echocardiogram. M-Mode echocardiography is not used to diagnose MVP. This is because the normal movement of the base of the heart can mimic or mask MVP.  The two- or three-dimensional echocardiogram allows measurement of leaflet thickness and displacement relative to the annulus.[8][9][10]

Prolapse is defined as mitral valve displacement more than 2 mm above mitral annulus in long axis view (parasternal or apical three chambers).  MVP is further subdivided into non-classic and classic based on the thickness of the mitral valve leaflets. In non-classic MVP, mitral valve leaflet thickness is 0 mm to 5 mm. In classic MVP, mitral valve leaflet thickness is more than 5 mm.

Classic MVP is further subdivided into symmetric and asymmetric based on the point at which leaflets tips join the mitral annulus. In symmetric form, leaflet tips meet at a common point on the annulus. In asymmetric form, one leaflet is displaced toward the atrium with respect to the other. 

Classic asymmetric MVP is further subdivided into flail and non-flail subtypes. In flail subtype, prolapse occurs when a leaflet tip turns outward, becoming concave toward the left atrium causing mitral valve deterioration. The flail leaflet varies from tip eversion to chordal rupture. Dissociation of leaflet and chordae tendineae results in unrestricted motion of the leaflet giving the name "flail leaflet."  The flail leaflet has a higher prevalence of mitral regurgitation than non-flail form.

Cardiovascular magnetic resonance imaging (CMRI) clinical modality has not been evaluated in MVP. CMR enables quantification of mitral regurgitation prior to mitral valve surgery. 

Sometimes, MVP is discovered incidentally on left ventriculography during cardiac catheterization. This is characterized by the displacement of mitral valve leaflets into the left atrium with late systolic mitral valve regurgitation. In such individuals, MVP should be evaluated by echocardiography. If there is discordant between clinical and echocardiographic findings on the severity of mitral valve regurgitation, then cardiac catheterization and left ventriculography would be useful. 

Treatment / Management

MVP patients with no symptoms often require no treatment.[11][12][13]

MVP patients with symptoms of dysautonomia (chest pain, palpitations), should be treated with beta-blockers such as propranolol. 

MVP with severe mitral regurgitation may benefit from mitral valve repair or mitral valve replacement. ACC/AHA guidelines recommend mitral valve repair before symptoms of congestive heart failure develop. 

Individuals with MVP are at high risk for bacterial endocarditis. Until 2007, the American Heart Association (AHA) recommended prescribing antibiotics before invasive procedures, including dental surgery. After that, AHA recommended that prophylaxis for dental procedures only should be advised for patients who other cardiac conditions which put them at the highest risk of adverse outcomes from infective endocarditis.

The association between MVP and a cerebral vascular event is low. The 2014 American Heart Association/American College of Cardiology (AHA/ACC) and 2012 European Society of Cardiology do not comment on antiplatelet/antithrombotic therapy in MVP. The 2006 ACC/AHA guidelines recommend aspirin for unexplained transient ischemic attacks in sinus rhythm with no atrial thrombi. Aspirin may be considered in sinus rhythm with echocardiographic evidence of high-risk MVP. Anticoagulation is recommended for systemic embolism or recurrent transient ischemic attacks (TIA) despite aspirin therapy. Anticoagulation is not recommended without systemic embolism, unexplained TIA, ischemic stroke, or atrial fibrillation.

Differential Diagnosis

MVP should be differentiated from other causes of mitral valve regurgitation.

Prognosis

The overall prognosis for MVP is benign.  Most asymptomatic individuals are not aware that they have MVP and do not require treatment. Complications associated with MVP include infective endocarditis, mitral valve regurgitation, arrhythmia (atrial fibrillation), transient ischemic event or systemic embolism. The major predictor of mortality in MVP is the degree of mitral valve regurgitation and ejection fraction. 

Complications

  • Progression to severe mitral regurgitation
  • Infective endocarditis
  • Atrial fibrillation
  • Stroke
  • Sudden death

Consultations

Cardiologist

Cardiothoracic surgeon

Deterrence and Patient Education

Patients who have been diagnosed with MVP should be advised to seek medical advice if the symptoms become worse. They have more benefit in surgical treatment before they develop congestive heart failure.

Pearls and Other Issues

 The prevalence of MVP in patients with Marfan's Syndrome is 91 %[14]

 

Enhancing Healthcare Team Outcomes

The management of MVP is multidisciplinary that includes a team of a cardiologist, cardiac nurse, primary care provider, and cardiac surgeon. Patients need to be educated that they have a benign disorder and the risk of complications is low. However, patients do need to be told about the symptoms of mitral regurgitation and endocarditis and when to present to the hospital. Patients should be encouraged to lead a healthy lifestyle, not smoke, exercise regularly and abstain from alcohol and caffeinated beverages.  For those who develop palpitations, a trial of beta blockers may prove to be useful.[15][16][17] (Level V)

Outcomes

The majority of patients with MVP have a normal life expectancy. About 3-10% of patients will have progression of the condition to severe mitral regurgitation. In general, patients over the age of 50 at diagnosis and normal left ventricular function have an excellent outcome, even if they do develop MR. Death is rare from MVP today. Even those who undergo repair or replacement of the valve, the outcomes are good to excellent. [18][19](Level V)


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Mitral Valve Prolapse - Questions

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What is the most common cause of non-ischemic mitral regurgitation?



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Which of the following is the most common valvular disorder in American children?



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A 30 year old female is found to have mitral valve prolapse with thin mitral valve leaflets and minimal regurgitation on echocardiogram. There are no other abnormalities. Select the most likely prognosis.



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A 22 year old female presents with atypical chest pain. Exam is remarkable for a midsystolic click but no murmur. Which of the following statements is accurate?



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A 17-year-old female is noted to have a late systolic apical murmur with preceding click. The rest of her physical is normal. Her mother used to take antibiotics before going to the dentist for a heart murmur but does not any longer. Select the most probable diagnosis.



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Which the following statement is true regarding mitral valve prolapse?



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A midsystolic click is most often heard in a patient with which of the following?



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What is often the result of mitral valve prolapse?

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You are seeing a 33-year old female in the clinic with complaints of palpitations that come and go on an irregular basis. She sometimes feels tired, gets dizzy, and develops panic attacks. She has no other past medical history and is on no medications. Auscultation reveals a mid-systolic click with a faint systolic murmur. She has been sent to the echo lab for further investigations. While waiting for the result, what treatment will be of benefit to her?



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Which of the following is the most common valvular disorder in American children?



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A 30 year old female is found to have mitral valve prolapse with thin mitral valve leaflets and minimal regurgitation on echocardiogram. There are no other abnormalities. Select the most likely prognosis.



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A middle-aged male presents with complaints of palpitations and vague chest pain. He claims that the symptoms have been ongoing for several months and come on when he performs any type of physical activity. He has a history of hypertension and arthritis. He is on enalapril and ibuprofen. He smokes and drinks socially. He had a remote appendectomy and is now on disability because of a leg injury. On auscultation, there is a late systolic murmur and no other sound. While blood work is pending, an echocardiogram is done which is shown below. There is displacement of the posterior leaflet with mild thickening. What is the next study of choice in this patient?

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Mitral Valve Prolapse - References

References

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Nalliah CJ,Mahajan R,Elliott AD,Haqqani H,Lau DH,Vohra JK,Morton JB,Semsarian C,Marwick T,Kalman JM,Sanders P, Mitral valve prolapse and sudden cardiac death: a systematic review and meta-analysis. Heart (British Cardiac Society). 2018 Sep 21     [PubMed]
Ayme-Dietrich E,Lawson R,Da-Silva S,Mazzucotelli JP,Monassier L, Serotonin contribution to cardiac valve degeneration: new insights for novel therapies? Pharmacological research. 2018 Sep 9     [PubMed]
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Kitkungvan D,Nabi F,Kim RJ,Bonow RO,Khan MA,Xu J,Little SH,Quinones MA,Lawrie GM,Zoghbi WA,Shah DJ, Myocardial Fibrosis in Patients With Primary Mitral Regurgitation With and Without Prolapse. Journal of the American College of Cardiology. 2018 Aug 21     [PubMed]
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Parwani P,Avierinos JF,Levine RA,Delling FN, Mitral Valve Prolapse: Multimodality Imaging and Genetic Insights. Progress in cardiovascular diseases. 2017 Nov - Dec     [PubMed]
Slipczuk L,Rafique AM,Davila CD,Beigel R,Pressman GS,Siegel RJ, The Role of Medical Therapy in Moderate to Severe Degenerative Mitral Regurgitation. Reviews in cardiovascular medicine. 2016     [PubMed]
Adams DH,Rosenhek R,Falk V, Degenerative mitral valve regurgitation: best practice revolution. European heart journal. 2010 Aug     [PubMed]
Suri RM,Aviernos JF,Dearani JA,Mahoney DW,Michelena HI,Schaff HV,Enriquez-Sarano M, Management of less-than-severe mitral regurgitation: should guidelines recommend earlier surgical intervention? European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 2011 Aug     [PubMed]
Scordo KA, Mitral valve prolapse syndrome health concerns, symptoms, and treatments. Western journal of nursing research. 2005 Jun     [PubMed]
Scordo KA, Factors associated with participation in a mitral valve prolapse support group. Heart     [PubMed]
Scordo KA, Mitral valve prolapse syndrome. Nonpharmacologic management. Critical care nursing clinics of North America. 1997 Dec     [PubMed]
Mazine A,Friedrich JO,Nedadur R,Verma S,Ouzounian M,Jüni P,Puskas JD,Yanagawa B, Systematic review and meta-analysis of chordal replacement versus leaflet resection for posterior mitral leaflet prolapse. The Journal of thoracic and cardiovascular surgery. 2018 Jan     [PubMed]
Bayer-Topilsky T,Suri RM,Topilsky Y,Marmor YN,Trenerry MR,Antiel RM,Mahoney DW,Schaff HV,Enriquez-Sarano M, Mitral Valve Prolapse, Psychoemotional Status, and Quality of Life: Prospective Investigation in the Current Era. The American journal of medicine. 2016 Oct     [PubMed]
Hayek E,Gring CN,Griffin BP, Mitral valve prolapse. Lancet (London, England). 2005 Feb 5-11;     [PubMed]
Hirata K,Triposkiadis F,Sparks E,Bowen J,Boudoulas H,Wooley CF, The Marfan syndrome: cardiovascular physical findings and diagnostic correlates. American heart journal. 1992 Mar;     [PubMed]

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