Mitral valve prolapse (MVP) is a billowing of mitral valve leaflets into the left atrium during systole. The most common cause is idiopathic myxomatous degeneration. MVP is usually benign, but complications include mitral regurgitation, endocarditis, chordal rupture, and possibly thromboembolism. MVP is usually asymptomatic in the absence of important regurgitation, although there are reports that some patients experience chest pain, dyspnea, dizziness, and palpitations. Signs include a crisp mid-systolic click, followed by a late systolic murmur if regurgitation is present. Diagnosis is by physical examination and echocardiography. Prognosis is excellent in the absence of significant regurgitation, but chordal rupture and endocarditis may occur. No specific treatment is necessary unless significant mitral regurgitation is present.

MVP is common; prevalence is 1 to 3% in otherwise normal populations, depending on the echocardiographic criteria used. Women and men are affected equally; onset usually follows the adolescent growth spurt.

Etiology

MVP is most often caused by myxomatous degeneration of the mitral valve and chordae tendineae. Degeneration is usually idiopathic, although it may be inherited in an autosomal dominant or, rarely, in an X-linked recessive fashion. Myxomatous degeneration may also be caused by connective tissue disorders (eg, Marfan syndrome, Ehlers-Danlos syndrome, adult polycystic kidney disease, osteogenesis imperfecta, pseudoxanthoma elasticum, SLE, polyarteritis nodosa) and muscular dystrophies. MVP is more common among patients with Graves disease, hypomastia, von Willebrand syndrome, sickle cell disease, and rheumatic heart disease. Myxomatous degeneration may also affect the aortic or tricuspid valve, resulting in aortic or tricuspid prolapse. Primary tricuspid regurgitation is much less common than secondary tricuspid regurgitation due to mitral regurgitation (MR).

MR due to MVP may occur in patients with apparently normal mitral valve leaflets (ie, nonmyxomatous) due to papillary muscle dysfunction or rheumatic chordal rupture. Transient MVP may occur when intravascular volume decreases significantly, as occurs in severe dehydration or sometimes during pregnancy (when the woman is recumbent and the gravid uterus compresses the inferior vena cava, reducing venous return).

MR is the most common complication of MVP. MR may be acute (due to ruptured chordae tendineae causing flail mitral valve leaflets) or chronic. Sequelae of chronic MR include heart failure and atrial fibrillation (AF) with thromboembolism. Whether MVP causes stroke independent of MR and AF is unclear. In addition, MR increases the risk of infective endocarditis, but MVP without MR does not.

Symptoms and Signs

Most patients are asymptomatic. Some experience nonspecific symptoms (eg, chest pain, dyspnea, palpitations, dizziness, near syncope, migraines, anxiety), thought to be due to poorly defined associated abnormalities in adrenergic signaling and sensitivity rather than to mitral valve pathology. In about one third of patients, emotional stress precipitates palpitations, which may be a symptom of benign arrhythmias (atrial premature beats, paroxysmal atrial tachycardia, ventricular premature beats, complex ventricular ectopy).

Occasionally, patients present with MR. Rarely, patients present with endocarditis (eg, fever, weight loss, thromboembolic phenomena) or stroke. Sudden death occurs in < 1%, most often resulting from ruptured chordae tendineae and flail mitral valve leaflets. Death due to a ventricular arrhythmia is rare.

Typically, MVP causes no visible or palpable cardiac signs. MVP alone often causes a crisp mid-systolic click as the subvalve apparatus abruptly tightens. The click is heard best with the diaphragm of the stethoscope over the left apex when the patient is in the left lateral decubitus position. MVP with MR causes a click with a late-systolic MR murmur. The click moves closer to the 1st heart sound (S₁) with maneuvers that decrease left ventricle (LV) size (eg, sitting, standing, Valsalva maneuver); the same maneuvers cause an MR murmur to appear or become louder and last longer. These effects occur because decreasing LV size causes papillary muscles and chordae tendineae to pull together more centrally beneath the valve, resulting in quicker, more forceful prolapse with earlier, more severe regurgitation. Conversely, squatting or isometric handgrip delays the S₁ click and shortens the MR murmur. The systolic click may be confused with the click of congenital aortic stenosis, which can be distinguished because it occurs very early in systole and does not move with postural or LV volume changes. Other findings include a systolic honk or whoop, thought to be caused by valvular leaflet vibration; these findings are usually transient and may vary with respiratory phase. An early diastolic opening snap caused by return of the prolapsed valve to its normal position is rarely heard.

Mitral Valve Prolapse (With Late-Systolic Click)

Mitral Valve Prolapse

Other physical findings associated with but not diagnostic of MVP include hypomastia, pectus excavatum, straight back syndrome, and a narrow anteroposterior chest diameter.

Diagnosis

• Echocardiography

Diagnosis is suggested clinically and confirmed by echocardiography. Thickened (≥ 5 mm), redundant mitral valve leaflets are thought to indicate more extensive myxomatous degeneration and greater risk of endocarditis and MR.

Holter monitoring and 12-lead ECG may be useful for documenting arrhythmias in patients with palpitations.

Prognosis

MVP is usually benign, but severe myxomatous degeneration of the valve can lead to MR. In patients with severe MR, incidence of LV or left atrium enlargement, arrhythmias (eg, AF), infective endocarditis, stroke, need for valve replacement, and death is about 2 to 4%/yr.

Treatment

• Usually none
• Sometimes β-blockers

MVP does not usually require treatment. β-Blockers may be used to relieve symptoms of excess sympathetic tone (eg, palpitations, migraines, dizziness) and to reduce risk of tachyarrhythmias, although no data support this practice. A typical regimen is atenolol 25 to 50 mg po once/day or propranolol 20 to 40 mg po bid. AF may require additional treatment (see Arrhythmias and Conduction Disorders: Treatment).

Treatment of MR depends on severity and associated left atrial and LV changes.

Antibiotic prophylaxis against endocarditis is no longer recommended. Anticoagulants to prevent thromboembolism are recommended only for patients with AF or prior transient ischemic attack or stroke.

Key Points

• MVP is most often caused by idiopathic myxomatous degeneration of the mitral valve and chordae tendineae.
• Mitral regurgitation (MR) is the most common complication.
• Heart sounds often include a sharp, mid-systolic click that occurs earlier with the Valsalva maneuver.
• Prognosis is usually benign unless MR develops, in which case there is increased risk of atrial fibrillation, stroke, and infective endocarditis,
• Treatment is not needed unless significant MR develops.

Last full review/revision November 2012 by Guy P. Armstrong