Mitral Valve Prolapse
- Mitral valve prolapse (MVP) is the billowing of one or both mitral valve leaflets into the left atrium (LA) during ventricular systole.
- MVP can be classified in different ways, including by etiology (primary or secondary; see “Etiology and Pathophysiology”) or by morphology (classic MVP and nonclassic MVP; see “Test Interpretation”).
- MVP is often asymptomatic and often has a benign clinical course; however, it may occasionally be associated with symptoms, such as palpitations, or complications, such as mitral regurgitation (MR) (see “Complications”).
- Synonym(s): systolic click-murmur syndrome, billowing mitral cusp syndrome, myxomatous mitral valve, floppy valve syndrome, redundant cusp syndrome, Barlow syndrome.
Etiology and Pathophysiology
- The pathophysiology of MVP typically involves myxomatous degeneration of the mitral valve leaflets, characterized by expansion of the valve spongiosa layer, structural alterations in collagen, and structurally abnormal chordae (1),(3).
- Papillary muscle or chordae disruption, dysfunction, or rupture may also cause MVP; this can occur without a process causing myxomatous degeneration or as part of the natural history of a process causing myxomatous degeneration.
- The etiology of MVP is multifactorial and includes the following (1):
- Primary MVP: sporadic or familial
- Secondary MVP
- “Syndromic” MVP: myxomatous degeneration associated with connective tissue disorders, for example Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, Loeys-Dietz syndrome
- Associated with congenital heart disease: atrial septal defect, Ebstein anomaly
- Papillary/chordae disruption, dysfunction or rupture: infarction, endocarditis, rheumatic fever, trauma, hypertrophic cardiomyopathy
- In primary MVP, both autosomal dominant and X-linked instances have been found.
- Connective tissue disorders, which often have a genetic basis, are associated with secondary MVP (see “Etiology and Pathophysiology”).
- Medical conditions implicated in the development of MVP include both heritable and sporadic congenital abnormalities as well as other disease processes. (See “Etiology and Pathophysiology.”)
- MVP is more common with leaner BMI (1).
Commonly Associated Conditions
- Conditions implicated in the development of MVP include both heritable and sporadic congenital abnormalities as well as other disease processes. (See “Etiology and Pathophysiology.”)
- MVP is implicated in the development of other medical conditions/complications, such as MR and stroke. (See “Complications.”)
- Some associated conditions that are less clearly either (i) a cause of MVP or (ii) caused by MVP include von Willebrand disease; primary hypomastia; thoracic skeletal abnormalities; and prolapse of the tricuspid, pulmonic, or aortic valves.
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