eMedicine Specialties > Emergency Medicine > Cardiovascular

Mitral Valve Prolapse

Author: Michael C Plewa, MD, Research Coordinator, Consulting Staff, Department of Emergency Medicine, Lucas County Emergency Physicians, Inc, and Saint Vincent Mercy Medical Center
Coauthor(s): Richard Worthington, MD, Assistant Clinical Professor, Program Instructor, Department of Emergency Medicine, St Vincent Mercy Medical Center
Contributor Information and Disclosures

Updated: Dec 22, 2008

Introduction

Background

Mitral valve prolapse (MVP) can occur in a multitude of disorders and, in most instances, it reflects a normal variant rather than a single disease process.

Despite years of research, the symptomatology and significance of MVP remain controversial. It was termed the disease of the decade in the 1980s, but now some consider it an interesting finding of dubious importance. Initial studies that reported associated symptoms of MVP as chest pain, dyspnea, anxiety, and panic, were probably flawed by recruitment bias. Recent studies imply that the incidence of MVP previously was overestimated by inaccurate echocardiographic diagnostic criteria and that associated symptoms, other than palpitations, are uncommon.1

Despite this, patients with MVP are at risk for endocarditis, stroke,2 mitral regurgitation (MR), mitral valve replacement (MVR) surgery,3 and sudden death.

Mitral valve prolapse. A patient with straight ba...

Mitral valve prolapse. A patient with straight back syndrome.

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Mitral valve prolapse. A patient with straight ba...

Mitral valve prolapse. A patient with straight back syndrome.


Pathophysiology

A myxomatous degeneration from collagen dissolution leads to excess mucopolysaccharides in the middle spongiosa layer of the mitral valve leaflets, resulting in stretching of the leaflets and the chordae tendineae.

Mitral valve prolapse (MVP) occurs when the left ventricular (LV) size is small in comparison to an enlarged mitral annulus, leaflets, or chordae tendineae, and it can be induced in healthy women with typical body habitus following dehydration that is reversed with rehydration. MVP resolves during pregnancy and following weight gain in anorexic patients.

Recent studies have shown that abnormalities of elastic fibers found in floppy mitral valves are related to genetic variants in fibrillin, one of the components of the microfibrils, as well as elastin and collagen I and II.4

A constellation of abnormalities (eg, increased sensitivity to adrenergic stimuli, increased catecholamines, abnormal beta-receptors, increased atrial natriuretic factor, renin-aldosterone dysregulation, decreased intravascular volume, magnesium deficiency)5 has been thought to lead to chest pain, dyspnea, fatigue, dizziness, near-syncope symptoms, and anxiety in a subset of patients with MVP.6,7

Cardiac manifestations include supraventricular arrhythmias, palpitations, mitral regurgitation, bacterial endocarditis, and sudden death. Chest pain may not be more common in patients with MVP than in the general population, and it may be attributed to myofascial syndromes, hyperventilation, coronary spasm, esophageal dysmotility, or gastroesophageal reflux.8

Mitral valve prolapse can result in cerebrovascular ischemia, which may be related to abnormal platelet activity or protein C or S deficiencies.

Frequency

United States

Mitral valve prolapse (MVP) can be identified by echocardiography in 3-4% of the general population, and it is identified in 7% of autopsies.

International

The worldwide incidence of MVP is similar to that in the United States.

Mortality/Morbidity

  • In general, mitral valve prolapse (MVP) is a benign disorder, but it may account for the majority of isolated cases of mitral regurgitation (MR), 90% of cases of ruptured chordae tendineae, 40% of strokes in young patients, and 10-15% of cases of endocarditis.
  • Those with structural abnormalities (ie, thickened, deformed, or redundant mitral valve leaflets) are more likely to suffer complications (eg, progressive MR, endocarditis, sudden death).
  • Cases of MVP with a murmur and not just an isolated click have a general mortality rate that is increased by 15-20%.

Race

Prevalence is similar among different ethnic groups.9

Sex

  • The female-to-male ratio is approximately 3:1.
  • Men older than 45 years have twice the risk of MR and endocarditis.

Age

Age of onset is 10-16 years.

  • Mitral valve prolapse (MVP) is uncommon before the adolescent growth spurt occurs. It usually is detected in young adulthood.
  • Although MVP is considered congenital, echocardiographic findings typically are absent in newborns.

Clinical

History

  • Mitral valve prolapse (MVP) usually is asymptomatic, nonprogressive, and benign.
  • Palpitations occur in 40% of MVP cases. This percentage excludes palpitations due to withdrawal syndromes (eg, alcohol, sedatives), intoxications (eg, cocaine, amphetamine, phencyclidine), or medication exposures (eg, caffeine, sympathomimetic, anticholinergic).
  • Chest pain and dyspnea previously were considered part of the MVP syndrome, but they are now felt to be no more common in cases of MVP than they are in the general population.10,1
  • Although controversial, anxiety and panic disorders may be more common in patients with MVP than the general population.11
  • Fatigue
  • Syncope/presyncope10
  • Orthostasis10

Physical

  • Thin aesthetic body habitus with narrow anteroposterior diameter12
  • Skeletal abnormalities (ie, pectus excavatum, straight back, kyphoscoliosis)
  • Supernumerary nipples in Asian Indians
  • Resting bradycardia and orthostatic hypotension
  • Cardiac auscultation
    • Apical, single or multiple, mid-to-late systolic clicks, which result from the tightening of the chordae tendineae or the redundant valve, can be heard.
    • An apical mid-to-late systolic murmur of crescendo, decrescendo, or constant nature can be heard, and the murmur continues to be heard in S2.
    • The click and the murmur change as the position changes (closer to S1 with diminished LV volume; closer to S2 with increased LV volume)
    • In the supine position, the click is late (ie, close to S2), and the murmur is brief.
    • In the standing position and during the Valsalva maneuver, the click is earlier (ie, close to S1), and the murmur is longer. This may identify a murmur that previously was not noted.
    • In the squatting position, the click is later (ie, closer to S2), and the murmur is shorter. The click and the murmur may even disappear.
    • The isometric handgrip exercise increases the intensity (ie, loudness) of the murmur without affecting the position.
    • The murmur should be distinguished from that of aortic stenosis (ie, early systolic, at base); pulmonic flow murmur (ie, short and early systolic, diminishes with Valsalva maneuver); hypertrophic cardiomyopathy (ie, diminishes with squatting and intensifies with standing and Valsalva maneuver); and mitral regurgitation (ie, holosystolic murmur with S3, enlarged and displaced point of maximal intensity [PMI]).
  • Mitral regurgitation
  • Autonomic dysfunction - Decreased heart rate variability and parasympathetic tone13,14
  • Neuroendocrine dysfunction
  • Ehlers-Danlos syndrome findings (eg, joint hypermobility, abnormal striae, bruising, distensibility of skin)
  • Osteogenesis imperfecta findings (eg, blue sclera)
  • Marfan syndrome findings (eg, scoliosis, straight back, pectus excavatum, arachnodactyly, arm span greater than body height)
  • Stickler syndrome findings (eg, kyphosis, scoliosis, mandibular hypoplasia, retinal detachment). Whether Stickler syndrome is associated with MVP is debatable.

Causes

Most cases of mitral valve prolapse are primary, idiopathic in nature, and expressed as an autosomal dominant trait that exhibits both sex- and age-dependent penetrance.15

More on Mitral Valve Prolapse

Overview: Mitral Valve Prolapse
Differential Diagnoses & Workup: Mitral Valve Prolapse
Treatment & Medication: Mitral Valve Prolapse
Follow-up: Mitral Valve Prolapse
Multimedia: Mitral Valve Prolapse
References
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References

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  3. Singh RG, Cappucci R, Kramer-Fox R, et al. Severe mitral regurgitation due to mitral valve prolapse: risk factors for development, progression, and need for mitral valve surgery. Am J Cardiol. Jan 15 2000;85(2):193-8. [Medline].

  4. Chou HT, Shi YR, Hsu Y. Association between fibrillin-1 gene exon 15 and 27 polymorphisms and risk of mitral valve prolapse. J Heart Valve Dis. Jul 2003;12(4):475-81. [Medline].

  5. Lichodziejewska B, Klos J, Rezler J, et al. Clinical symptoms of mitral valve prolapse are related to hypomagnesemia and attenuated by magnesium supplementation. Am J Cardiol. Mar 15 1997;79(6):768-72. [Medline].

  6. Scordo KA. Mitral valve prolapse syndrome: interventions for symptom control. Dimens Crit Care Nurs. Jul-Aug 1998;17(4):177-86. [Medline].

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  8. Kao CH, Tsai SC, Hsieh JF, et al. Radionuclide esophageal transit test to detect esophageal disorders in patients with mitral valve prolapse. Nuklearmedizin. 2000;39(4):92-6. [Medline].

  9. Theal M, Sleik K, Anand S. Prevalence of mitral valve prolapse in ethnic groups. Can J Cardiol. Apr 2004;20(5):511-5. [Medline].

  10. Devereux RB, Kramer-Fox R, Kligfield P. Mitral valve prolapse: causes, clinical manifestations, and management. Ann Intern Med. Aug 15 1989;111(4):305-17. [Medline].

  11. Tamam L, Ozpoyraz N, San M, Bozkurt A. Association between idiopathic mitral valve prolapse and panic disorder. Croat Med J. Dec 2000;41(4):410-6. [Medline].

  12. Raggi P, Callister TQ, Lippolis NJ, Russo DJ. Is mitral valve prolapse due to cardiac entrapment in the chest Cavity? A CT view. Chest. Mar 2000;117(3):636-42. [Medline].

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  14. Kochiadakis GE, Parthenakis FI, Zuridakis EG, et al. Is there increased sympathetic activity in patients with mitral valve prolapse?. Pacing Clin Electrophysiol. Nov 1996;19(11 Pt 2):1872-6. [Medline].

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  16. Ozkan M, Kaymaz C, Dinckal H, et al. Single-photon emission computed tomographic myocardial perfusion imaging in patients with mitral valve prolapse. Am J Cardiol. Feb 15 2000;85(4):516-8, A11. [Medline].

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  27. Kitlinski M, Stepniewski M, Nessler J. Is magnesium deficit in lymphocytes a part of the mitral valve prolapse syndrome?. Magnes Res. Mar 2004;17(1):39-45. [Medline].

  28. La Vecchia L, Ometto R, Centofante P, et al. Arrhythmic profile, ventricular function, and histomorphometric findings in patients with idiopathic ventricular tachycardia and mitral valve prolapse: clinical and prognostic evaluation. Clin Cardiol. Oct 1998;21(10):731-5. [Medline].

  29. Leung DY, Dawson IG, Thomas JD, Marwick TH. Accuracy and cost-effectiveness of exercise echocardiography for detection of coronary artery disease in patients with mitral valve prolapse. Am Heart J. Dec 1997;134(6):1052-7. [Medline].

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Further Reading

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Contributor Information and Disclosures

Author

Michael C Plewa, MD, Research Coordinator, Consulting Staff, Department of Emergency Medicine, Lucas County Emergency Physicians, Inc, and Saint Vincent Mercy Medical Center
Michael C Plewa, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, Physicians for Social Responsibility, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Richard Worthington, MD, Assistant Clinical Professor, Program Instructor, Department of Emergency Medicine, St Vincent Mercy Medical Center
Richard Worthington, MD is a member of the following medical societies: American College of Emergency Physicians, Ohio State Medical Association, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Medical Editor

Robert M McNamara, MD, FAAEM, Chair and Professor, Department of Emergency Medicine, Temple University School of Medicine
Robert M McNamara, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American Medical Association, Pennsylvania Medical Society, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Paul Blackburn, DO, FACOEP, FACEP, Program Director, Department of Emergency Medicine, Maricopa Medical Center; Assistant Professor, Department of Surgery, University of Arizona
Paul Blackburn, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Medical Association, and Arizona Medical Association
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

David FM Brown, MD, Assistant Professor, Division of Emergency Medicine, Harvard Medical School; Vice Chair, Department of Emergency Medicine, Massachusetts General Hospital
David FM Brown, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

 
 
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