Elsevier

The Lancet

Volume 365, Issue 9458, 5–11 February 2005, Pages 507-518
The Lancet

Seminar
Mitral valve prolapse

https://doi.org/10.1016/S0140-6736(05)17869-6Get rights and content

Summary

Mitral valve prolapse is a common valvular abnormality that is the most common cause of severe non-ischaemic mitral regurgitation in the USA. The overall prognosis of patients with mitral valve prolapse is excellent, but a small subset will develop serious complications, including infective endocarditis, sudden cardiac death, and severe mitral regurgitation. We present a comprehensive review of mitral valve prolapse, examining normal mitral anatomy, the clinical and echocardiographic features of mitral valve prolapse, and the pathophysiology and genetics of the disorder. We discuss the contemporary management of both asymptomatic and symptomatic prolapse, with particular attention to the timing and technique of surgical repair. We conclude that echocardiography is the method of choice for diagnosing mitral valve prolapse, that clinical and echocardiographic features can predict which patients with prolapse are at highest risk for complications, and that mitral valve repair is the treatment of choice for symptomatic 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

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

References (150)

  • WR Mills et al.

    Clinical, echocardiographic, and biomechanical differences in mitral valve prolapse affecting one or both leaflets

    Am J Cardiol

    (2002)
  • D Lax et al.

    Mild dehydration induces echocardiographic signs of mitral valve prolapse in healthy females with prior normal cardiac findings

    Am Heart J

    (1992)
  • D Levy et al.

    Prevalence and clinical features of mitral valve prolapse

    Am Heart J

    (1987)
  • JM Flack et al.

    Anthropometric and physiologic correlates of mitral valve prolapse in a biethnic cohort of young adults: the CARDIA study

    Am Heart J

    (1999)
  • K Hirata et al.

    The Marfan syndrome: cardiovascular physical findings and diagnostic correlates

    Am Heart J

    (1992)
  • SD Lima et al.

    Relation of mitral valve prolapse to left ventricular size in Marfan's syndrome

    Am J Cardiol

    (1985)
  • NV Strahan et al.

    Inheritance of the mitral valve prolapse syndrome. Discussion of a three-dimensional penetrance model

    Am J Med

    (1983)
  • S Disse et al.

    Mapping of a first locus for autosomal dominant myxomatous mitral-valve prolapse to chromosome 16p11.2-p12.1

    Am J Hum Genet

    (1999)
  • LA Freed et al.

    A locus for autosomal dominant mitral valve prolapse on chromosome 11p15.4

    Am J Hum Genet

    (2003)
  • HT Chou et al.

    Association between COL3A1 collagen gene exon 31 polymorphism and risk of floppy mitral valve/mitral valve prolapse

    Int J Cardiol

    (2004)
  • JF Nasuti et al.

    Fibrillin and other matrix proteins in mitral valve prolapse syndrome

    Ann Thorac Surg

    (2004)
  • R Nascimento et al.

    Is mitral valve prolapse a congenital or acquired disease?

    Am J Cardiol

    (1997)
  • AJ Weis et al.

    Anatomic explanation of mobile systolic clicks: implications for the clinical and echocardiographic diagnosis of mitral valve prolapse

    Am Heart J

    (1995)
  • A Zuppiroli et al.

    Natural history of mitral valve prolapse

    Am J Cardiol

    (1995)
  • A Zuppiroli et al.

    A family study of anterior mitral leaflet thickness and mitral valve prolapse

    Am J Cardiol

    (1998)
  • MD Cheitlin et al.

    ACC/AHA/ASE 2003 guideline update for the clinical application of echocardiography—summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/ASE Committee to Update the 1997 Guidelines for the Clinical Application of Echocardiography)

    J Am Coll Cardiol

    (2003)
  • A Carpentier

    Cardiac valve surgery—the “French correction”

    J Thorac Cardiovasc Surg

    (1983)
  • GP Foster et al.

    Accurate localization of mitral regurgitant defects using multiplane transesophageal echocardiography

    Ann Thorac Surg

    (1998)
  • E Agricola et al.

    Multiplane transesophageal echocardiography performed according to the guidelines of the American Society of Echocardiography in patients with mitral valve prolapse, flail, and endocarditis: diagnostic accuracy in the identification of mitral regurgitant defects by correlation with surgical findings

    J Am Soc Echocardiogr

    (2003)
  • AS Omran et al.

    Intraoperative transesophageal echocardiography accurately predicts mitral valve anatomy and suitability for repair

    J Am Soc Echocardiogr

    (2002)
  • RB Devereux et al.

    Relation between clinical features of the mitral prolapse syndrome and echocardiographically documented mitral valve prolapse

    J Am Coll Cardiol

    (1986)
  • DD Savage et al.

    Mitral valve prolapse in the general population. 2. Clinical features: the Framingham Study

    Am Heart J

    (1983)
  • CM Pratt et al.

    Exercise testing in women with chest pain. Are there additional exercise characteristics that predict true positive test results?

    Chest

    (1989)
  • DY Leung et al.

    Accuracy and cost-effectiveness of exercise echocardiography for detection of coronary artery disease in patients with mitral valve prolapse

    Am Heart J

    (1997)
  • M Ozkan et al.

    Single-photon emission computed tomographic myocardial perfusion imaging in patients with mitral valve prolapse

    Am J Cardiol

    (2000)
  • M Greenspan et al.

    Exercise myocardial scintigraphy with 201-thallium. Use in patients with mitral valve prolapse without associated coronary artery disease

    Chest

    (1980)
  • A Pasternac et al.

    Increased plasma catecholamine levels in patients with symptomatic mitral valve prolapse

    Am J Med

    (1982)
  • A Anwar et al.

    Altered beta adrenergic receptor function in subjects with symptomatic mitral valve prolapse

    Am J Med Sci

    (1991)
  • T Szombathy et al.

    Angiotensin II type 1 receptor gene polymorphism and mitral valve prolapse syndrome

    Am Heart J

    (2000)
  • E Chesler et al.

    Normal catecholamine and hemodynamic responses to orthostatic tilt in subjects with mitral valve prolapse. Correlation with psychologic testing

    Am J Med

    (1985)
  • RB Devereux et al.

    Complications of mitral valve prolapse. Disproportionate occurrence in men and older patients

    Am J Med

    (1986)
  • LA Freed et al.

    Mitral valve prolapse in the general population: the benign nature of echocardiographic features in the Framingham Heart Study

    J Am Coll Cardiol

    (2002)
  • MF Stoddard et al.

    Exercise-induced mitral regurgitation is a predictor of morbid events in subjects with mitral valve prolapse

    J Am Coll Cardiol

    (1995)
  • SW MacMahon et al.

    Mitral valve prolapse and infective endocarditis

    Am Heart J

    (1987)
  • SW MacMahon et al.

    Risk of infective endocarditis in mitral valve prolapse with and without precordial systolic murmurs

    Am J Cardiol

    (1987)
  • JM Steckelberg et al.

    Risk factors for infective endocarditis

    Infect Dis Clin North Am

    (1993)
  • RB Devereux et al.

    Cost-effectiveness of infective endocarditis prophylaxis for mitral valve prolapse with or without a mitral regurgitant murmur

    Am J Cardiol

    (1994)
  • CJ Frary et al.

    Clinical and health care cost consequences of infective endocarditis in mitral valve prolapse

    Am J Cardiol

    (1994)
  • AS Dajani et al.

    Prevention of bacterial endocarditis: recommendations by the American Heart Association

    J Am Dent Assoc

    (1997)
  • WA Pocock et al.

    Sudden death in primary mitral valve prolapse

    Am Heart J

    (1984)
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