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Mitral Valve Prolapse Last Updated: November 6, 2006 |
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| Synonyms and related keywords: mitral valve prolapse, MVP, myxomatous mitral valve, floppy mitral valve syndrome, Barlow's syndrome, Barlow syndrome, billowing mitral cusp syndrome, systolic click-murmur syndrome, myxomatous mitral valve, redundant cusp syndrome, irritable heart, effort syndrome, soldier's heart, isolated mitral regurgitation, MVP syndrome
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AUTHOR INFORMATION
| Section 1 of 9   |
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| Author: Bhavik V Thakkar, MD, Associate Faculty, Department of Medical Education, Abbott Northwestern Hospital; Consulting Staff, Department of Medicine, Regency Hospital Coauthor(s): Alan D Forker, MD, Professor of Medicine, Program Director of Cardiovascular Fellowship, Department of Medicine, Mid America Heart Institute, University of Missouri at Kansas City School of Medicine; Co-Director, Lipid Diabetes Research Center, Saint Luke's Hospital; Adam E Schussheim, MD, Consulting Staff, Department of Internal Medicine, Bridgeport Hospital of the Yale-New Haven Medical Center |
| 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, and
Minnesota Medical Association |
| Editor(s): Justin D Pearlman, MD, ME, PhD, MA, Director of Dartmouth Advanced Imaging Center, Professor of Medicine, Professor of Radiology, Adjunct Professor, Thayer Bioengineering and Computer Science, Dartmouth-Hitchcock Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine;
Marschall S Runge, MD, PhD, Marion Covington Distinguished Professor of Medicine, Vice Dean for Clinical Affairs, School of Medicine, Chairman, Department of Medicine, University of North Carolina at Chapel Hill;
Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital;
and Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice |
Disclosure
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INTRODUCTION
| Section 2 of 9   |
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Background: 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 prognosis. 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. Pathophysiology: Most patients with MVP are asymptomatic, and the natural history is benign. However, when large, floppy valves result in severe mitral regurgitation, especially due to ruptured chordae tendinea, mitral valve surgery or repair may be necessary. Myxomatous proliferation is the most common pathologic basis for MVP, which can lead to myxomatous degeneration of the loose spongiosa and fragmentation of collagen fibrils. Disruption of the endothelium may predispose patients to infectious endocarditis and thromboembolic complications. However, worth reemphasis is that the vast majority of patients with MVP have only a minor derangement of the structure of the mitral valve that is usually clinically insignificant. Frequency:
- In the US: MVP is thought to be inherited with increased expression of the gene in female individuals by a margin of 2:1. The most common form of inheritance is autosomal dominant, whereas the relatively uncommon X-chromosomal form is linked with myxomatous degeneration.
In the absence of rigorous echocardiographic criteria in the 1970-1980s, MVP was overdiagnosed with a reported prevalence of 5-15%. One study reported an incidence of 38% among teenagers.
Levine et al, who described the 3-dimensional saddle shape of the mitral leaflets and annulus, reported that the 2-dimensional echocardiographic characterizations of prolapse, especially on the parasternal long-axis view, are most specific and help in minimizing false-positive diagnoses (Levine, 1987).
Data from the community-based Framingham study demonstrated that MVP syndrome occurred in only 2.4% of the population.
Also worth mentioning is that MVP commonly occurs with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum. A striking statistic is that 91% of patients with Marfan syndrome have MVP due to the increased redundancy of the mitral leaflets and apparatus due to myxomatous degeneration.
Mortality/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. Men older than 50 years have the most serious consequences of hemodynamically significant mitral regurgitation.
Age: MVP has been observed in all ages and in both sexes.
History: MVP is usually diagnosed from the physical examination, with the classic auscultatory finding of a mid-to-late systolic click, or it is incidentally diagnosed during echocardiography. MVP is also discovered when complications of MVP manifest. Most patients are asymptomatic. Symptomatic patients with MVP are separated into 2 categories: those with symptoms related to autonomic dysfunction and those with symptoms related to the progression of mitral regurgitation. - Symptoms related to autonomic dysfunction are usually associated with genetic inheritance and include the following:
- Neuropsychiatric symptoms
- Symptoms related to progression of mitral regurgitation include the following:
- Paroxysmal nocturnal dyspnea (PND)
- Progressive signs of congestive heart failure (CHF)
- ECG usually is normal, but can show nonspecific ST-segment and T wave abnormalities especially in leads II, III, aVF.
- MVP is also commonly seen in patients with inheritable connective tissue disorders.
Physical: Clinical characteristics are typically benign in young women, whereas men older than 50 years tend to have serious consequence of mitral regurgitation. - Common general physical features associated with MVP include the following:
- Low body weight or body mass index (BMI)
- 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, which is present due to the leaflets prolapsing into the left atrium resulting in tensing of the mitral valve apparatus. It may or may not be followed by a high-pitched, crescendo, late-systolic murmur at the cardiac apex.
- The midsystolic click can vary in intensity and timing, primarily depending on left ventricular volume.
- End-diastolic volume can be reduced by performing a Valsalva maneuver or by having the patient stand. These maneuvers result in an early click close to the first heart sound and a prolonged murmur. In the supine position, especially with the legs raised for increased venous return, left ventricular volume is increased, resulting in a click late in systole, close to the second heart sound, and a shortened murmur.
- Patients with MVP most frequently have symptoms of autonomic dysfunction, including easy fatigability, dizziness, and atypical chest pain. This pain is perhaps related to papillary muscle strain (ie, excessive pulling on the left ventricular wall with prolapsed leaflets in the left atrium).
Causes: MVP usually occurs as an isolated entity. As previously mentioned, it also commonly occurs with heritable disorders of connective tissue. MVP has also been described in association with coronary artery disease, hypertrophic cardiomyopathy, and rheumatic heart disease.
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DIFFERENTIALS
| Section 4 of 9 |
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Mitral Regurgitation
Other Problems to be Considered:
Consider all inheritable connective tissue disorders mentioned previously, with emphasis on Marfan syndrome because of its increased frequency. All causes of mitral regurgitation should be considered but if only a mid-systolic click and/or if a crescendo, late-systolic murmur is present; in such case, the diagnosis is almost always MVP. If only a late systolic murmur without a click is present, papillary muscle dysfunction secondary to coronary artery disease is a possibility. If only a mid-to-late systolic click is present, mitral valve prolapse can be confused with mitral stenosis; that is, the systolic click is misinterpreted as a diastolic opening snap. |
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Imaging Studies:
- Diagnostic criteria
- Perloff et al set the stage for accurately diagnosing MVP by expanding the diagnostic standards to include clinical and echocardiographic criteria (Perloff, 1986; Perloff, 1989).
- In a Framingham Heart Study, Freed et al historically described echocardiographic criteria for MVP as classic versus nonclassic (Freed, 1999).
- Use of the parasternal long-axis view Levine et al described may reduce the number of false-positive diagnoses of MVP (Levine, 1987).
- Findings
- Classic MVP: The parasternal long-axis view may show superior displacement of the mitral leaflets of >2 mm during systole into the left atrium, with a thickness of at least 5 mm.
- Nonclassic MVP: Displacement is >2 mm, with a maximal thickness of <5 mm.
- Other: Other echocardiographic findings that should be considered as criteria are leaflet thickening, redundancy, annular dilatation, and chordal elongation.
- Contrast ventriculography: This study can also help in defining MVP with or without mitral regurgitation. However, with the advent of echocardiography, contrast ventriculography is rarely necessary.
- Chest radiography: Radiographs may demonstrate the progression from asymptomatic to severe mitral regurgitation with the development of cardiomegaly secondary to left atrial and left ventricular dilatation and with evidence of heart failure.
Medical Care: - Asymptomatic patients with minimal disease
- These patients should be strongly reassured of their benign prognosis.
- They should undergo initial echocardiography for risk stratification. If no clinically significant mitral regurgitation is identified with thin leaflets, clinical examinations and echocardiographic studies can be scheduled every 3-5 years.
- These patients are encouraged to pursue a normal, unrestricted lifestyle, including vigorous exercise.
- Patients with symptoms of autonomic dysfunction
- A trial of beta-blockers for symptomatic relief can be tried.
- They are also recommended to abstain from stimulants such as caffeine, alcohol, and cigarettes. An ambulatory 24-hour monitor may be useful to detect supraventricular and/or ventricular arrhythmias. If frequent ventricular ectopy or VT is identified, electrophysiology studies may be indicated to identify the small risk of sudden cardiac death.
- Patients with evidence of or progression to severe mitral regurgitation
- Close follow-up and referral for surgical repair are indicated early, before left ventricular dilatation and systolic dysfunction develop.
- Asymptomatic patients with moderate-to-severe mitral regurgitation and left ventricular enlargement, especially those with atrial fibrillation and/or pulmonary hypertension, should undergo surgery soon, before left ventricular dysfunction becomes poor.
- If the physician is unsure if the patient is asymptomatic, a treadmill stress test for exercise tolerance should be performed. That is, have the patient demonstrate that he or she can walk vigorously without symptoms.
- Patients with MVP and neurologic findings
- After atrial fibrillation and left atrial thrombus are excluded, these patients should be given daily aspirin therapy at a dosage of 80-325 mg/d.
- Daily aspirin is also recommended for patients with transient ischemic attacks (TIAs) and patients <65 years with atrial fibrillation and no history of mitral regurgitation, hypertension or heart failure (ie, lone atrial fibrillation).
- Patients should stop smoking and using oral contraceptives to help prevent a hypercoagulable state.
- Warfarin should be used when patients older than 65 years 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.
- Patients with a mid-systolic click and late-systolic mitral regurgitation murmur
- Seriously consider antibiotic prophylaxis in these patients, including those with increased leaflet thickening or redundancy, left atrial enlargement, and left ventricular dilatation, even in the absence of correlated clinical findings.
- Most clinicians and textbook authors usually consider an isolated mid-to-late systolic click without a murmur a low-risk condition. However, if the echocardiographic findings of redundancy and leaflet thickness are impressive, some cardiologists still offer prophylactic antibiotics.
Surgical Care: See the surgical management discussion in Mitral Regurgitation.
Further Outpatient Care:
- Depending on its severity, patients with MVP may be monitored regularly with history taking, physical examination, and echocardiography when indicated.
- If symptoms or physical findings change during outpatient care, echocardiography or a combination of studies may be indicated.
Complications:
- Severe mitral regurgitation
- This is the most common complication and the 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 individuals, history of hypertension, increased BMI, increased mitral valve thickness or redundancy, or left atrial and left ventricular dilatation.
- The main mechanism for increased risk includes leaflet thickness or redundancy and the severity of mitral regurgitation resulting in a turbulent flow state.
- The risk increases 3- to 8-fold.
- The main predictors are age older than 50 years, male sex, history of hypertension, increased BMI, left atrial and ventricular enlargement, or increased mitral valve thickness or redundancy.
- If an isolated mid-to-late systolic click is present, it is generally thought to be low risk, but universal agreement is lacking.
- 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 (1999) 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 have again suggested 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.
Prognosis:
- 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.
Patient Education:
- Patients should receive education regarding their prognosis along with the signs and symptoms of disease progression.
- Of emphasis, most patients with MVP have a benign prognosis, but the risk of ruptured chordae and/or clinically significant mitral regurgitation, infective endocarditis, embolic TIA or stroke, and rare sudden death must also be discussed.
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MISCELLANEOUS
| Section 8 of 9 |
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Medical/Legal Pitfalls:
- Strict diagnostic criteria should be used to offset the number of false-positive diagnoses of MVP that occurred in the 1970-1980s.
- Previous echocardiographic studies produced undue anxiety, particularly in asymptomatic patients.
- Once again, the vast majority of patients with MVP should be strongly reassured of their benign prognosis.
- Underdiagnosis and failure to follow up patients with clinically significant MVP can be damaging.
- When clinically significant MR develops or progresses, irreversible left ventricular dysfunction may occur.
- Also, endocarditis can be a subtle illness that makes prompt diagnosis difficult.
Special Concerns:
- In many patients, MVP was diagnosed on the basis of imprecise echocardiographic criteria used in the 1970-1980s.
- In addition, being told that they have a heart condition can greatly affect patients' self-perceptions. Care should be taken to explain MVP syndrome in detail so that patients understand that the natural history of MVP is usually benign.
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BIBLIOGRAPHY
| Section 9 of 9 |
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