Definition and diagnosis of mitral valve prolapse
- Sorin Pislaru, MD, PhD
Sorin Pislaru, MD, PhD
- Associate Professor of Medicine
- Division of Cardiovascular Diseases
- Mayo Clinic
- Maurice Enriquez-Sarano, MD
Maurice Enriquez-Sarano, MD
- Professor of Medicine
- Mayo Medical School
Mitral valve prolapse (MVP) is a common cause of mitral regurgitation (MR). Although most patients with MVP have mild, trivial, or no MR , it is the most common cause of surgical MR in developed countries . Other potential complications include infective endocarditis and arrhythmias. The diagnosis of MVP is suspected on physical examination and confirmed by echocardiography.
The definition, classification, etiology, pathology, and diagnosis of MVP will be reviewed here. Other aspects of MVP and associated MR are discussed separately. (See "Nonarrhythmic complications of mitral valve prolapse" and "Arrhythmic complications of mitral valve prolapse" and "Natural history of chronic mitral regurgitation in mitral valve prolapse and flail mitral leaflet" and "Mitral valve prolapse syndrome".)
The prevalence of mitral valve prolapse (MVP) in the general population varies among studies, mostly due to variable criteria used for diagnosis. Reports published early in the development of echocardiography suggested high MVP prevalence, 4 to 10 percent [3-5] and even close to 20 percent in selected populations. These early reports are now considered inaccurate because echocardiographic criteria for diagnosis were not yet fully developed and lacked specificity.
Using currently accepted definition of MVP, the Framingham Heart Study reported an overall prevalence of 2.4 percent . Individuals with classic MVP (leaflet thickness ≥5 mm; 1.3 percent) and non-classic MVP (leaflet thickness <5mm; 1.1 percent) had similar age and sex distributions. In another population-based study (Cardia), the prevalence of MVP in 4136 young adults was only 0.6 percent . In a Canadian study of 972 patients, MVP prevalence was similar in three ethnic groups (2.7 percent in South Asian, 3.1 percent in European, and 2.2 percent in Chinese) . MVP patients were leaner and had a greater degree of mitral regurgitation than the general population [6,7]. It is unclear whether prevalence differences between studies are age-related and should be interpreted as demonstrating a link between aging and MVP prevalence.
MVP may be slightly more common in women than in men. In the Framingham study, there was a nonsignificant trend toward a female preponderance among those with MVP (59.5 versus 52.7 percent in those without MVP) ; in the larger Olmsted county study, 64 percent of individuals with MVP were women .
- Freed LA, Benjamin EJ, Levy D, 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; 40:1298.
- Luxereau P, Dorent R, De Gevigney G, et al. Aetiology of surgically treated mitral regurgitation. Eur Heart J 1991; 12 Suppl B:2.
- Procacci PM, Savran SV, Schreiter SL, Bryson AL. Prevalence of clinical mitral-valve prolapse in 1169 young women. N Engl J Med 1976; 294:1086.
- Darsee JR, Mikolich JR, Nicoloff NB, Lesser LE. Prevalence of mitral valve prolapse in presumably healthy young men. Circulation 1979; 59:619.
- Hickey AJ, Wolfers J, Wilcken DE. Mitral-valve prolapse: prevalence in an Australian population. Med J Aust 1981; 1:31.
- Freed LA, Levy D, Levine RA, et al. Prevalence and clinical outcome of mitral-valve prolapse. N Engl J Med 1999; 341:1.
- Flack JM, Kvasnicka JH, Gardin JM, et al. Anthropometric and physiologic correlates of mitral valve prolapse in a biethnic cohort of young adults: the CARDIA study. Am Heart J 1999; 138:486.
- Theal M, Sleik K, Anand S, et al. Prevalence of mitral valve prolapse in ethnic groups. Can J Cardiol 2004; 20:511.
- Avierinos JF, Gersh BJ, Melton LJ 3rd, et al. Natural history of asymptomatic mitral valve prolapse in the community. Circulation 2002; 106:1355.
- Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118:e523.
- Grewal J, Suri R, Mankad S, et al. Mitral annular dynamics in myxomatous valve disease: new insights with real-time 3-dimensional echocardiography. Circulation 2010; 121:1423.
- Clavel MA, Mantovani F, Malouf J, et al. Dynamic phenotypes of degenerative myxomatous mitral valve disease: quantitative 3-dimensional echocardiographic study. Circ Cardiovasc Imaging 2015; 8.
- Levine RA, Handschumacher MD, Sanfilippo AJ, et al. Three-dimensional echocardiographic reconstruction of the mitral valve, with implications for the diagnosis of mitral valve prolapse. Circulation 1989; 80:589.
- Levine RA, Triulzi MO, Harrigan P, Weyman AE. The relationship of mitral annular shape to the diagnosis of mitral valve prolapse. Circulation 1987; 75:756.
- BARLOW JB, POCOCK WA. The significance of late systolic murmurs and mid-late systolic clicks. Md State Med J 1963; 12:76.
- Barlow JB, Bosman CK, Pocock WA, Marchand P. Late systolic murmurs and non-ejection ("mid-late") systolic clicks. An analysis of 90 patients. Br Heart J 1968; 30:203.
- Devereux RB, Kramer-Fox R, Shear MK, et al. Diagnosis and classification of severity of mitral valve prolapse: methodologic, biologic, and prognostic considerations. Am Heart J 1987; 113:1265.
- Perloff JK, Child JS, Edwards JE. New guidelines for the clinical diagnosis of mitral valve prolapse. Am J Cardiol 1986; 57:1124.
- Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2014; 63:e57.
- de Marchena E, Badiye A, Robalino G, et al. Respective prevalence of the different carpentier classes of mitral regurgitation: a stepping stone for future therapeutic research and development. J Card Surg 2011; 26:385.
- Morganroth J, Jones RH, Chen CC, Naito M. Two dimensional echocardiography in mitral, aortic and tricuspid valve prolapse. The clinical problem, cardiac nuclear imaging considerations and a proposed standard for diagnosis. Am J Cardiol 1980; 46:1164.
- Ogawa S, Hayashi J, Sasaki H, et al. Evaluation of combined valvular prolapse syndrome by two-dimensional echocardiography. Circulation 1982; 65:174.
- Bon Tempo CP, Ronan JA Jr, de Leon AC Jr, Twigg HL. Radiographic appearance of the thorax in systolic click-late systolic murmur syndrome. Am J Cardiol 1975; 36:27.
- Kelly RE, Goretsky MJ, Obermeyer R, et al. Twenty-one years of experience with minimally invasive repair of pectus excavatum by the Nuss procedure in 1215 patients. Ann Surg 2010; 252:1072.
- Pickering NJ, Brody JI, Barrett MJ. von Willebrand syndromes and mitral-valve prolapse; linked mesenchymal dysplasias. N Engl J Med 1981; 305:131.
- Rosenberg CA, Derman GH, Grabb WC, Buda AJ. Hypomastia and mitral-valve prolapse. Evidence of a linked embryologic and mesenchymal dysplasia. N Engl J Med 1983; 309:1230.
- Grau JB, Pirelli L, Yu PJ, et al. The genetics of mitral valve prolapse. Clin Genet 2007; 72:288.
- Strahan NV, Murphy EA, Fortuin NJ, et al. Inheritance of the mitral valve prolapse syndrome. Discussion of a three-dimensional penetrance model. Am J Med 1983; 74:967.
- Scheele W, Allen HN, Kraus R, et al. Familial prevalence and genetic transmission of mitral valve prolapse. Circulation 1977; 56 (Suppl. 3):111.
- Monteleone PL, Fagan LF. Possible X-linked congenital heart disease. Circulation 1969; 39:611.
- Lardeux A, Kyndt F, Lecointe S, et al. Filamin-a-related myxomatous mitral valve dystrophy: genetic, echocardiographic and functional aspects. J Cardiovasc Transl Res 2011; 4:748.
- Glesby MJ, Pyeritz RE. Association of mitral valve prolapse and systemic abnormalities of connective tissue. A phenotypic continuum. JAMA 1989; 262:523.
- Attias D, Stheneur C, Roy C, et al. Comparison of clinical presentations and outcomes between patients with TGFBR2 and FBN1 mutations in Marfan syndrome and related disorders. Circulation 2009; 120:2541.
- Attenhofer Jost CH, Connolly HM, O'Leary PW, et al. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 2005; 80:361.
- Lucas RV Jr, Edwards JE. The floppy mitral valve. Curr Probl Cardiol 1982; 7:1.
- Cole WG, Chan D, Hickey AJ, Wilcken DE. Collagen composition of normal and myxomatous human mitral heart valves. Biochem J 1984; 219:451.
- Barber JE, Kasper FK, Ratliff NB, et al. Mechanical properties of myxomatous mitral valves. J Thorac Cardiovasc Surg 2001; 122:955.
- Adams DH, Carabello BA, Castillo J. Mitral valve regurgitation. In: Hurst's The Heart, 13th ed, Fuster V, Walsh RA, Harrington RA. (Eds), McGraw-Hill, New York 2010.
- Eriksson MJ, Bitkover CY, Omran AS, et al. Mitral annular disjunction in advanced myxomatous mitral valve disease: echocardiographic detection and surgical correction. J Am Soc Echocardiogr 2005; 18:1014.
- Carpentier A, Lacour-Gayet F, Camilleri J, et al. Fibroelastic dysplasia of the mitral valve. An anatomical clinical entity. Circulation 1980; 62 (Suppl. III):111.
- Prunotto M, Caimmi PP, Bongiovanni M. Cellular pathology of mitral valve prolapse. Cardiovasc Pathol 2010; 19:e113.
- Fornes P, Heudes D, Fuzellier JF, et al. Correlation between clinical and histologic patterns of degenerative mitral valve insufficiency: a histomorphometric study of 130 excised segments. Cardiovasc Pathol 1999; 8:81.
- Heidenreich PA, Bear J, Browner W, Foster E. The clinical impact of echocardiography on antibiotic prophylaxis use in patients with suspected mitral valve prolapse. Am J Med 1997; 102:337.
- Devereux RB, Kramer-Fox R, Kligfield P. Mitral valve prolapse: causes, clinical manifestations, and management. Ann Intern Med 1989; 111:305.
- Barron JT, Manrose DL, Liebson PR. Comparison of auscultation with two-dimensional and Doppler echocardiography in patients with suspected mitral valve prolapse. Clin Cardiol 1988; 11:401.
- Weis AJ, Salcedo EE, Stewart WJ, et al. Anatomic explanation of mobile systolic clicks: implications for the clinical and echocardiographic diagnosis of mitral valve prolapse. Am Heart J 1995; 129:314.
- Malcolm AD. Mitral valve prolapse associated with other disorders. Casual coincidence, common link, or fundamental genetic disturbance? Br Heart J 1985; 53:353.
- Monin JL, Dehant P, Roiron C, et al. Functional assessment of mitral regurgitation by transthoracic echocardiography using standardized imaging planes diagnostic accuracy and outcome implications. J Am Coll Cardiol 2005; 46:302.
- Pepi M, Tamborini G, Maltagliati A, et al. Head-to-head comparison of two- and three-dimensional transthoracic and transesophageal echocardiography in the localization of mitral valve prolapse. J Am Coll Cardiol 2006; 48:2524.
- Hien MD, Großgasteiger M, Rauch H, et al. Experts and beginners benefit from three-dimensional echocardiography: a multicenter study on the assessment of mitral valve prolapse. J Am Soc Echocardiogr 2013; 26:828.
- Mantovani F, Clavel MA, Vatury O, et al. Cleft-like indentations in myxomatous mitral valves by three-dimensional echocardiographic imaging. Heart 2015; 101:1111.
- Enriquez-Sarano M, Avierinos JF, Messika-Zeitoun D, et al. Quantitative determinants of the outcome of asymptomatic mitral regurgitation. N Engl J Med 2005; 352:875.
- Pizarro R, Bazzino OO, Oberti PF, et al. Prospective validation of the prognostic usefulness of brain natriuretic peptide in asymptomatic patients with chronic severe mitral regurgitation. J Am Coll Cardiol 2009; 54:1099.
- Kang DH, Kim JH, Rim JH, et al. Comparison of early surgery versus conventional treatment in asymptomatic severe mitral regurgitation. Circulation 2009; 119:797.
- Han Y, Peters DC, Salton CJ, et al. Cardiovascular magnetic resonance characterization of mitral valve prolapse. JACC Cardiovasc Imaging 2008; 1:294.
- DEFINITION AND CLASSIFICATION
- PRIMARY MVP
- Sporadic primary MVP
- Familial primary MVP
- SECONDARY MVP
- Secondary MVP associated with connective tissue disorders
- Secondary MVP due to mechanisms other than myxomatous degeneration
- CLINICAL MANIFESTATIONS
- MVP syndrome
- Physical examination
- - Cardiac examination
- - Other physical examination findings
- 2D echocardiography
- 3D echocardiography
- Doppler imaging
- OTHER IMAGING
- Cardiovascular magnetic resonance
- Left ventricular angiography
- Differential diagnosis
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS