Mitral Valve Prolapse
- Author: Bhavik V Thakkar, MD; Chief Editor: Richard A Lange, MD, MBA more...
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.
Signs and Symptoms
Most patients with MVP are asymptomatic. Symptoms are related to one of the following:
Progression of mitral regurgitation
An associated complication (ie, stroke, endocarditis, or arrhythmia)
Symptoms related to autonomic dysfunction are usually associated with genetically inherited MVP and include the following:
Atypical chest pain
Syncope or presyncope
Symptoms related to progression of mitral regurgitation include the following:
Paroxysmal nocturnal dyspnea (PND)
Progressive signs of chronic heart failure (CHF)
Common general physical features associated with MVP include the following:
Asthenic body habitus
Low body weight or BMI
Scoliosis or kyphosis
Hypermobility of the joints
Arm 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.
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 < 5 mm
Other echocardiographic findings that should be considered as criteria are leaflet thickening, redundancy, annular dilatation, and chordal elongation
See Workup for more detail.
For purposes of treatment, patients with MVP can be divided into the following categories:
Asymptomatic patients with minimal disease
Patients with symptoms of autonomic dysfunction
Patients with evidence of progression to severe mitral regurgitation
Patients with neurologic findings
Patients with a mid-systolic click and late-systolic mitral regurgitation murmur
Treatment measures for asymptomatic patients with minimal disease
Strong reassurance of the benign prognosis
Initial 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 relief
Abstinence from stimulants such as caffeine, alcohol, and cigarettes
An 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 develop
Surgery 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/d
Cessation of smoking and oral contraceptive use to prevent a hypercoagulable state
Warfarin 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 redundancy
Antibiotic 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
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.
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.
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.
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.
MVP has been observed in all ages.
Most patients with MVP are asymptomatic and have a benign course.
Patients with high-risk characteristics and/or progressive mitral regurgitation are at increased risk for complications.
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.
Complications of MVP include the following:
Severe mitral regurgitation
Sudden cardiac death and cerebrovascular ischemic events
Severe mitral regurgitation
This is the most common complication of MVP and leading cause of isolated mitral regurgitation requiring mitral valve surgery in the United States.
Severe mitral regurgitation is most frequently due to rupture of the chordae tendineae.
The risk increases with the following factors: patients older than 50 years, male sex, history of hypertension, increased BMI, increased mitral valve thickness or redundancy, and left atrial and left ventricular dilatation.
The main mechanism for increased risk is a turbulent flow state due to leaflet thickness or redundancy and mitral regurgitation. The risk of endocarditis increases 3- to 8-fold with MVP.
The main predictors are age older than 50 years, male sex, history of hypertension, increased BMI, left atrial and ventricular enlargement, and increased mitral valve thickness or redundancy.
If an isolated mid-to-late systolic click is present (eg, no murmur), antibiotic prophylaxis is not usually recommended unless the echocardiogram demonstrates significant leaflet redundancy and/or thickness.
Sudden cardiac death and cerebrovascular ischemic events
The association between sudden cardiac death and MVP is not well understood. Data suggest that MVP alone does result in excessive atrial or ventricular arrhythmias, which are most likely due to autonomic dysfunction. Patients with these findings have been said to have MVP syndrome.
The risk is increased when patients have evidence of left ventricular dilatation and dysfunction, severe mitral regurgitation, and increased mitral leaflet thickness or redundancy.
In the presence of QT prolongation and frequent ventricular ectopy, especially nonsustained ventricular or sustained ventricular tachycardia, an electrophysiologic study may be indicated to quantitate the risk of inducible ventricular tachycardia and/or ventricular fibrillation and sudden arrhythmic death.
In regard to cerebrovascular ischemic events, recent studies yielded mixed findings in terms of the association between the increased prevalence of cerebrovascular events and MVP in young patients without evidence of cerebrovascular disease. Gilon et al describes the lack of an association between MVP and stroke in young patients in a large case-control study. The hypothesized mechanism is the formation of platelet fibrin thrombi on the denuded, damaged myxomatous valves resulting in embolization. Data suggest that the prevalence of this mechanism is based on the degree of mitral regurgitation.
The major risk factors for cerebrovascular events include age older than 50 years, thickened mitral valve leaflets, atrial fibrillation, and a need for mitral valve surgery.
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