Mitral Valve Prolapse (MVP)
Mid-systolic click ± late systolic murmur from billowing mitral leaflet — usually benign, occasionally progresses to severe MR.
Also known as: MVP, Barlow disease, click-murmur syndrome, floppy mitral valve
Overview
Systolic displacement of one or both mitral leaflets ≥2 mm above the mitral annular plane into the left atrium, with or without leaflet thickening, as seen on the parasternal long-axis echo view.
Epidemiology
Affects roughly 2-3% of the general population with equal sex distribution when defined by strict echocardiographic criteria. Most patients are asymptomatic. Severe MR develops in a small minority, often after decades.
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Risk factors
- Connective tissue disease: Marfan syndrome, Ehlers-Danlos, Loeys-Dietz, osteogenesis imperfecta
- Family history (autosomal dominant in some kindreds)
- Female sex (symptomatic but not anatomic predominance)
- Skeletal anomalies: pectus excavatum, scoliosis, straight back syndrome
- Polycystic kidney disease
Pathophysiology
Myxomatous degeneration of the mitral leaflets and chordae (excess proteoglycan deposition) produces redundant, floppy tissue. During systole, leaflets bow into the LA; chordal stretch or rupture can produce mitral regurgitation. The classic click marks sudden chordal tension; the murmur follows as MR develops.
Clinical presentation
Symptoms
- Most patients asymptomatic — incidental auscultatory or echo finding
- Atypical chest pain (non-exertional, sharp, brief)
- Palpitations (premature beats, occasional SVT)
- Anxiety, fatigue, exercise intolerance (MVP syndrome — controversial association)
- Progressive MR symptoms: dyspnea on exertion, orthopnea (late finding)
Signs / physical exam
- Mid-to-late systolic click best heard at apex
- Late systolic murmur if MR present; becomes holosystolic as MR worsens
- Maneuvers that DECREASE LV size (Valsalva strain phase, standing) bring the click EARLIER and lengthen the murmur
- Maneuvers that INCREASE LV size (squatting, leg raise, handgrip) push the click LATER and shorten the murmur
- Thin, tall body habitus with skeletal features in connective tissue disease
Differential diagnosis
- Hypertrophic cardiomyopathy — Systolic ejection murmur that increases with Valsalva and standing; LVH on ECG/echo; SAM of mitral valve
- Aortic stenosis — Crescendo-decrescendo systolic ejection murmur at RUSB with carotid radiation; no click
- Tricuspid valve prolapse — Right-sided click and murmur, increase with inspiration
- Pericardial knock or split S2 — Timing and respiratory variation differ; no leaflet abnormality on echo
- Functional / non-pathologic systolic clicks — No prolapse on echo, no MR, asymptomatic
Diagnostic workup
Labs
- Generally none if isolated and asymptomatic
- Connective tissue evaluation if syndromic features
- TSH if palpitations
Imaging
- Transthoracic echo — diagnostic; documents leaflet displacement ≥2 mm above annulus, leaflet thickness, MR severity, LV/LA size
- Transesophageal echo for preoperative planning or unclear anatomy
- Cardiac MRI for severity quantification in selected patients
- ECG: usually normal; may show inferior T-wave inversion or ventricular ectopy; rarely prolonged QT
Diagnostic algorithm
| Maneuver | LV preload/size | Effect on MVP click and murmur |
|---|---|---|
| Valsalva (strain) | Decreased | Click EARLIER, murmur LONGER |
| Standing from squat | Decreased | Click EARLIER, murmur LONGER |
| Squatting / passive leg raise | Increased | Click LATER, murmur SHORTER |
| Sustained handgrip (↑afterload) | Increased | Click LATER, murmur LOUDER if MR present |
| Amyl nitrite (↓afterload) | Decreased | Click EARLIER, murmur LONGER but softer |
Treatment
First-line
- Reassurance and lifestyle counseling for asymptomatic isolated MVP without significant MR
- Avoid stimulants (caffeine, nicotine, decongestants) if palpitations symptomatic
- Beta-blocker (metoprolol, atenolol, propranolol) for symptomatic palpitations or atypical chest pain
- Routine endocarditis prophylaxis NOT recommended for isolated MVP (per current ACC/AHA guidelines)
- Serial echo every 3-5 years if mild MR; every 6-12 months if moderate-severe MR
Second-line / adjunct
- Mitral valve repair (preferred over replacement) — Class I for severe symptomatic MR, or asymptomatic severe MR with LVEF 30-60% or LV end-systolic diameter ≥40 mm
- Mitral valve replacement when repair not feasible
- Anticoagulation only if atrial fibrillation, prior embolic event, or LV thrombus — not for MVP alone
Complications
- Progressive mitral regurgitation requiring surgery
- Chordae tendineae rupture → acute severe MR and pulmonary edema
- Infective endocarditis (low absolute risk but elevated vs general population)
- Atrial fibrillation
- Ventricular arrhythmia and rare sudden cardiac death (especially bileaflet MVP with mitral annular disjunction)
PANCE pearls
- Mnemonic: Valsalva (decreased preload) brings the MVP click CLOSER to S1 — opposite of most other murmurs.
- Routine antibiotic endocarditis prophylaxis is NOT indicated for MVP per modern guidelines.
- Bileaflet MVP plus mitral annular disjunction (MAD) carries an elevated risk of ventricular arrhythmia.
- Repair is favored over replacement for degenerative MVP — better survival and ventricular function.
- MVP is the most common cause of chronic non-ischemic MR in developed countries.
References
- ACC/AHA 2020 VHD — 2020 ACC/AHA Guideline for the Management of Patients with Valvular Heart Disease (Otto et al., Circulation 2021)
- AHA 2007 IE Prophylaxis — Prevention of Infective Endocarditis (Wilson et al., Circulation 2007)
- Framingham — Prevalence and Clinical Outcome of Mitral Valve Prolapse (Freed et al., NEJM 1999)
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