eMedicine Specialties > Cardiology > Valvular Heart Disease

Mitral Valve Prolapse

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, MidAmerica Heart Institute, University of Missouri at Kansas City School of Medicine; Director, Outpatient Lipid Diabetes Research Center, MidAmerica Heart Institute of Saint Luke's Hospital; Adam E Schussheim, MD, Consulting Staff, Department of Internal Medicine, Bridgeport Hospital of the Yale-New Haven Medical Center
Contributor Information and Disclosures

Updated: Jul 30, 2008

Introduction

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 course. 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 their natural history is benign. However, when large, floppy valves or ruptured chordae tendinea result in severe mitral regurgitation, mitral valve surgery or repair may be necessary. Myxomatous proliferation is the most common pathologic basis for MVP, and it can lead to myxomatous degeneration of the loose spongiosa and fragmentation of the collagen fibrils. Disruption of the endothelium may predispose patients to infectious endocarditis and thromboembolic complications. However, the vast majority of patients with MVP have only a minor derangement of the mitral valve structure that is usually clinically insignificant.

Frequency

United States

MVP is thought to be inherited with increased expression of the gene in female individuals (2:1). The most common form of inheritance is autosomal dominant, but X-linked inheritance has been described.

MVP commonly occurs with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum. In fact, 90% of patients with Marfan syndrome have MVP due to the increased redundancy of the mitral leaflets and apparatus that occur as a result of myxomatous degeneration.

In the 1970s and 1980s, MVP was overdiagnosed because of the absence of rigorous echocardiographic criteria, with a reported prevalence of 5-15%. Subsequently, Levine et al reported that the 2-dimensional echocardiographic characterizations of prolapse, especially on the parasternal long-axis view, are most specific for the diagnosis of MVP.1 Use of these criteria prevent overdiagnosis.

Data from the community-based Framingham study demonstrated that MVP syndrome occurred in only 2.4% of the population.

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. The most serious consequences of hemodynamically significant mitral regurgitation occur in men older than 50 years.

Age

MVP has been observed in all ages.

Clinical

History

Mitral valve prolapse (MVP) is often diagnosed from the physical examination, when the classic auscultatory finding of a mid-to-late systolic click and/or murmur is appreciated. Alternatively, it may be incidentally diagnosed during routine echocardiography or discovered when complications of MVP manifest.

Most patients are asymptomatic. Symptomatic patients with MVP are separated into 3 categories: (1) those with symptoms related to autonomic dysfunction; (2) those with symptoms related to the progression of mitral regurgitation; and (3) those with symptoms that occur as a result of an associated complication (ie, stroke, endocarditis, or arrhythmia).

  • Symptoms related to autonomic dysfunction are usually associated with genetically inherited MVP and include the following:
    • Anxiety
    • Panic attacks
    • Arrhythmias
    • Exercise intolerance
    • Palpitations
    • Atypical chest pain
    • Fatigue
    • Orthostasis
    • Syncope or presyncope
    • Neuropsychiatric symptoms
  • Symptoms related to progression of mitral regurgitation include the following:
    • Fatigue
    • Dyspnea
    • Exercise intolerance
    • Orthopnea
    • 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:
    • Asthenic body habitus
    • Low body weight or body mass index (BMI)
    • Straight-back syndrome
    • Scoliosis or kyphosis
    • Pectus excavatum
    • 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, mid-to-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, which is 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 diastolic volume is increased, resulting in a click later in systole 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 a secundum atrial septal defect.

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
References
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Further Reading

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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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Contributor Information and Disclosures

Author

Bhavik V Thakkar, MD, Associate Faculty, Department of Medical Education, Abbott Northwestern Hospital; Consulting Staff, Department of Medicine, Regency Hospital
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
Disclosure: Nothing to disclose.

Coauthor(s)

Alan D Forker, MD, Professor of Medicine, Program Director of Cardiovascular Fellowship, MidAmerica Heart Institute, University of Missouri at Kansas City School of Medicine; Director, Outpatient Lipid Diabetes Research Center, MidAmerica Heart Institute of Saint Luke's Hospital
Alan D Forker, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, American Society of Hypertension, and Phi Beta Kappa
Disclosure: Research Grant Grant/research funds Hospital contracts to do research; I am a hospital employee with no personal profit; Speakers Bureau Honoraria Speaking and teaching

Adam E Schussheim, MD, Consulting Staff, Department of Internal Medicine, Bridgeport Hospital of the Yale-New Haven Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Justin D Pearlman, MD, PhD, ME, MA, Director of Dartmouth Advanced Imaging Center, Professor of Medicine, Professor of Radiology, Adjunct Professor, Thayer Bioengineering and Computer Science, Dartmouth-Hitchcock Medical Center
Justin D Pearlman, MD, PhD, ME, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Marschall S Runge, MD, PhD, Charles and Anne Sanders Distinguished Professor of Medicine, Chairman of Medicine, Vice Dean for Clinical Affairs, Chairman, Department of Medicine, University of North Carolina at Chapel Hill School of Medicine
Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association
Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD, E Cowles Andrus Professor of Cardiology, Professor of Medicine, Johns Hopkins University School of Medicine
Richard A Lange, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, and Association of Subspecialty Professors
Disclosure: Nothing to disclose.

 
 
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