To identify the published data on mitral valve prolapse, we searched MEDLINE and PubMed from 1966 to present and selected articles that included the keywords “myxomatous” and “mitral valve prolapse”. We restricted our search to articles in English that were about human beings, and included review articles and original research publications. Letters to the editor, commentaries, and case reports were excluded. Further data for the histopathological and biomechanical properties of myxomatous
SeminarMitral valve prolapse
Section snippets
Defining mitral valve prolapse
Initially mitral valve prolapse was defined as a late systolic murmur associated with “billowing” or prolapse of one or both of the mitral leaflets into the left atrium. With increased use of two-dimensional echocardiography, the diagnosis of mitral valve prolapse became much more prevalent—as high as 38% among teenage girls.5 In part, this overdiagnosis was due to the erroneous assumption that the mitral valve was planar; thus, any view that showed excursion of the leaflets superior to the
Cause and pathology
Mitral valve prolapse is a multifactorial valvular abnormality that can be caused by histological abnormalities of valvular tissue, geometric disparities between the left ventricle and mitral valve, or various connective tissue disorders. Leaflet thickening and redundancy, known as myxomatous degeneration, is the most common and clinically important of these abnormalities. Normal valve tissue is divided into three layers: an inner ventricularis layer, the middle spongiosa, and the outer
Genetics
Most cases of myxomatous mitral valve prolapse are sporadic. However, a familial basis for the condition has long been recognised, with an autosomal dominant mode of inheritance, variable penetrance influenced by age and sex, and a marked heterogeneity of clinical presentation even between affected members of the same family.39, 40, 41, 42 In 1999, the first locus for autosomal dominant myxomatous mitral valve prolapse, MMVP1, was mapped to chromosome 16p11.2-p12.1.43 In 2003, Freed and
Diagnosis
Physical examination and two-dimensional echocardiography are the diagnostic standards for mitral valve prolapse. The classic auscultatory finding is a dynamic, mid-to-late systolic click, frequently associated with a high-pitched, late systolic murmur. Specific manoeuvres—including Valsalva, squatting, and leg raises—are occasionally useful to demonstrate that the click moves within systole as left ventricular volume and loading conditions change. Reduction of end-diastolic volume (eg,
Mitral valve prolapse syndrome
Mitral valve prolapse is usually diagnosed on the basis of a classic physical examination, discovered incidentally on an echocardiogram done for another reason, or found once complications of the prolapse are manifest. Various symptoms (including atypical chest pain, exertional dyspnoea, palpitations, syncope, and anxiety) and clinical findings (including low blood pressure, leaner build, and electrocardiographic repolarisation abnormalities) have been associated with mitral valve prolapse and
Managing mitral valve prolapse
Mitral valve prolapse is equally common in men and women, although men seem to have a higher incidence of complications.3, 52, 75 Nevertheless, most patients with mitral valve prolapse have an excellent prognosis with an expected survival similar to that of the general population.11, 76, 77 Most patients do not develop symptoms or other significant echocardiographic abnormalities.76 Most asymptomatic patients with mitral valve prolapse can be followed conservatively. Neither their activity
Timing of surgery
Valve surgery is clearly warranted for patients with symptomatic severe mitral regurgitation, and those who are asymptomatic but have left ventricular enlargement (end-systolic diameter >45 mm) or even mildly reduced systolic function (ejection fraction <60%).52 Additionally, severe mitral regurgitation accompanied by atrial fibrillation or pulmonary hypertension carries an AHA/ACC Class IIa recommendation for valve surgery (table 3).52 Mitral valve repair offers several important advantages
Mitral valve repair techniques
The surgical approach, feasibility, and durability of repair are highly dependent on the mechanism of valve dysfunction. Transoesophageal echocardiography can accurately determine which leaflet is involved and, in the setting of bileaflet prolapse, whether significant anterior chordal pathology is present.59, 138 Frequently, more than one segment or scallop is involved, although posterior leaflet dysfunction predominates. A large study assessing patients with transoesophageal echocardiography
Operative risk and durability
In experienced centres, mitral valve repair can be done with an operative mortality of less than 1%.116, 139 Despite the potential presence of residual myxomatous tissue and chordae following valve repair, the rate of reoperation is surprisingly low, with 93% freedom from reoperation at 10 years and 80% at 20 years.139, 149 Compared with mitral valve replacement, repair provides a similar long-term durability and improved long-term survival; life expectancy of patients is similar to that of the
Search strategy and selection criteria
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Intraoperative transesophageal echocardiography accurately predicts mitral valve anatomy and suitability for repair
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Accuracy and cost-effectiveness of exercise echocardiography for detection of coronary artery disease in patients with mitral valve prolapse
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Exercise myocardial scintigraphy with 201-thallium. Use in patients with mitral valve prolapse without associated coronary artery disease
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Increased plasma catecholamine levels in patients with symptomatic mitral valve prolapse
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Risk factors for infective endocarditis
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Cost-effectiveness of infective endocarditis prophylaxis for mitral valve prolapse with or without a mitral regurgitant murmur
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Clinical and health care cost consequences of infective endocarditis in mitral valve prolapse
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Prevention of bacterial endocarditis: recommendations by the American Heart Association
J Am Dent Assoc
Sudden death in primary mitral valve prolapse
Am Heart J
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