Friday, December 27, 2013

Practice Essentials

Mitral valve prolapse (MVP) is the most common valvular abnormality, affecting approximately 2-6% of the population in the United States. MVP usually has a benign course, but it occasionally leads to serious complications, including clinically significant mitral regurgitation, infective endocarditis, sudden cardiac death, and cerebrovascular ischemic events.

Essential update: More accurate imaging of mitral valve prolapse with 3D echocardiography

Real-time 3-dimensional transesophageal echocardiography (3D TEE) is superior to 2-dimensional TEE for quantifying MVP in patients with severe mitral regurgitation, according to a study of 102 patients with severe mitral regurgitation due to MVP, mitral valve flail, or both. Compared with 3D TEE, 2D TEE underestimated the width of the prolapse and the leaflet gap. In addition, because of the complicated anatomy of the mitral valve, 2D imaging could not detect the largest prolapse gap and width.[1]

Signs and Symptoms

Most patients with MVP are asymptomatic. Symptoms are related to one of the following:

Autonomic dysfunctionProgression of mitral regurgitationAn associated complication (ie, stroke, endocarditis, or arrhythmia)

Symptoms related to autonomic dysfunction are usually associated with genetically inherited MVP and include the following:

AnxietyPanic attacksArrhythmiasExercise intolerancePalpitationsAtypical chest painFatigueOrthostasisSyncope or presyncopeNeuropsychiatric symptoms

Symptoms related to progression of mitral regurgitation include the following:

FatigueDyspneaExercise intoleranceOrthopneaParoxysmal nocturnal dyspnea (PND)Progressive signs of chronic heart failure (CHF)

Common general physical features associated with MVP include the following:

Asthenic body habitusLow body weight or BMIStraight-back syndromeScoliosis or kyphosisPectus excavatumHypermobility of the jointsArm span greater than height (which may be indicative of Marfan syndrome)

The classic auscultatory finding is a mid-to-late systolic click. It may or may not be followed by a high-pitched, mid-to-late systolic murmur at the cardiac apex. These can vary with the following maneuvers:

A Valsalva maneuver or having the patient stand result in an early click, which is close to the first heart sound, and a prolonged murmur The supine position, especially with the legs raised, results in a click later in systole and a shortened murmur

See Clinical Presentation for more detail.

Diagnosis

Findings on echocardiography are as follows:

Classic MVP: The parasternal long-axis view shows > 2 mm superior displacement of the mitral leaflets into the left atrium during systole, with a leaflet thickness of at least 5 mm Nonclassic MVP: Displacement is > 2 mm, with a maximal leaflet thickness of

Other echocardiographic findings that should be considered as criteria are leaflet thickening, redundancy, annular dilatation, and chordal elongation

See Workup for more detail.

Management

For purposes of treatment, patients with MVP can be divided into the following categories:

Asymptomatic patients with minimal diseasePatients with symptoms of autonomic dysfunctionPatients with evidence of progression to severe mitral regurgitationPatients with neurologic findingsPatients with a mid-systolic click and late-systolic mitral regurgitation murmur

Treatment measures for asymptomatic patients with minimal disease

Strong reassurance of the benign prognosisInitial echocardiography for risk stratification; if no clinically significant mitral regurgitation and thin leaflets are observed, clinical examinations and echocardiographic studies can be scheduled every 3-5 years Encouragement to pursue a normal, unrestricted lifestyle, including vigorous exercise

Treatment measures for patients with symptoms of autonomic dysfunction

A trial of beta-blockers for symptomatic reliefAbstinence from stimulants such as caffeine, alcohol, and cigarettesAn ambulatory 24-hour monitor may be useful to detect supraventricular and/or ventricular arrhythmias

Treatment measures for patients with evidence of or progression to severe mitral regurgitation

Close follow-up and early referral for surgical repair, before left ventricular dilatation and systolic dysfunction developSurgery before left ventricular function deteriorates in asymptomatic patients with moderate-to-severe mitral regurgitation and left ventricular enlargement, especially those with atrial fibrillation and/or pulmonary hypertension Treadmill stress test for exercise tolerance if the physician is unsure the patient is asymptomatic

Treatment measures for patients with neurologic findings

After atrial fibrillation and left atrial thrombus are excluded, daily aspirin therapy at a dosage of 80-325 mg/dCessation of smoking and oral contraceptive use to prevent a hypercoagulable stateWarfarin therapy for patients older than 65 years who have atrial fibrillation, especially if they have associated risk factors of a previous stroke or TIA, clinically significant valvular heart disease, hypertension, diabetes, left atrial enlargement, or a history and/or findings of heart failure

Treatment measures for patients with a mid-systolic click and late-systolic mitral regurgitation murmur

Consider antibiotic prophylaxis, including for patients with increased leaflet thickening or redundancyAntibiotic prophylaxis is not recommended for the patient with an isolated mid-to-late systolic click without a murmur, unless the echocardiogram demonstrates significant leaflet redundancy and/or thickness

See Treatment and Medication for more detail.

NextBackground

Mitral valve prolapse (MVP) is the most common valvular abnormality, affecting approximately 2-6% of the population in the United States. MVP usually results in a benign course. However, it occasionally leads to serious complications, including clinically significant mitral regurgitation, infective endocarditis, sudden cardiac death, and cerebrovascular ischemic events. MVP is also the most common cause of isolated mitral regurgitation in the United States, and it is the most common reason for mitral valve surgery.

PreviousNextPathophysiology

Most patients with MVP are asymptomatic, and their natural history is benign. However, when large, floppy valves or ruptured chordae tendinea result in severe mitral regurgitation, mitral valve surgery or repair may be necessary. Myxomatous proliferation is the most common pathologic basis for MVP, and it can lead to myxomatous degeneration of the loose spongiosa and fragmentation of the collagen fibrils. Disruption of the endothelium may predispose patients to infectious endocarditis and thromboembolic complications. However, the vast majority of patients with MVP have only a minor derangement of the mitral valve structure that is usually clinically insignificant.

PreviousNextFrequencyUnited States

MVP is thought to be inherited with increased expression of the gene in female individuals (2:1). The most common form of inheritance is autosomal dominant, but X-linked inheritance has been described.

MVP commonly occurs with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum. In fact, 90% of patients with Marfan syndrome have MVP due to the increased redundancy of the mitral leaflets and apparatus that occur as a result of myxomatous degeneration.

In the 1970s and 1980s, MVP was overdiagnosed because of the absence of rigorous echocardiographic criteria, with a reported prevalence of 5-15%. Subsequently, Levine et al reported that the 2-dimensional echocardiographic characterizations of prolapse, especially on the parasternal long-axis view, are most specific for the diagnosis of MVP. Use of these criteria prevent overdiagnosis.

Data from the community-based Framingham study demonstrated that MVP syndrome occurred in only 2.4% of the population.

PreviousNextMortality/Morbidity

Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe mitral regurgitation) have increased cardiac morbidity and mortality rates, especially if reduced left ventricular systolic function is present.

See Complications.

Sex

MVP occurs more frequently in young women than in men. The most serious consequences of hemodynamically significant mitral regurgitation occur in men older than 50 years.

Age

MVP has been observed in all ages.

PreviousProceed to Clinical Presentation  Contributor Information and DisclosuresAuthor

Bhavik V Thakkar, MD  Medical Director, Internal Medicine Hospitalist, AppleCare Medical Group
Bhavik V Thakkar, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Heart Association, American Medical Association, American Stroke Association, Minnesota Medical Association, and Society for Vascular Medicine and Biology
Disclosure: Nothing to disclose.

Coauthor(s)

Adam E Schussheim, MD  Consulting Staff, Department of Internal Medicine, Bridgeport Hospital of the Yale-New Haven Medical Center
Disclosure: Nothing to disclose.

Specialty Editor Board

Justin D Pearlman, MD, ME, PhD, FACC, MA  Chief, Division of Cardiology, Director of Cardiology Consultative Service, Director of Cardiology Clinic Service, Director of Cardiology Non-Invasive Laboratory, Director of Cardiology Quality Program KMC, Dartmouth-Hitchcock Medical Center, Dartmouth Medical School
Justin D Pearlman, MD, ME, PhD, FACC, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America
Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine
Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association
Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Private Practice
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD  Professor and Executive Vice Chairman, Department of Medicine, Director, Office of Educational Programs, University of Texas Health Science Center at San Antonio
Richard A Lange, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, and Association of Subspecialty Professors
Disclosure: Nothing to disclose.

Additional Contributors

Alan D Forker, MD Professor of Medicine, University of Missouri at Kansas City School of Medicine; Director, Outpatient Lipid Diabetes Research, MidAmerica Heart Institute of St Luke's Hospital

Disclosure: Nothing to disclose.

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