Cardiovascular · PANCE / PANRE

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

How bedside maneuvers shift the MVP click-murmur complex.

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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