History and exam

Key diagnostic factors

common

presence of risk factors

Key factors include connective tissue diseases (such as Marfan's syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum), positive family history, and slim body type.

mid-systolic click

The combination of click and murmur is specific but not sensitive for diagnosis.[34]

Changes in timing with dynamic manoeuvres help to differentiate mitral valve prolapse from other causes. With a decrease in preload (standing, Valsalva) or decrease in afterload (amyl nitrate administration), the click and murmur occur earlier in systole. Conversely, with an increase in left ventricular volume (squatting) or increase in afterload (handgrip) the click and murmur occur later.

late-systolic murmur

The duration and intensity of murmur vary with loading conditions.

The murmur may progress to holosystolic with progressive severity of mitral regurgitation. With a decrease in preload (standing, Valsalva) or decrease in afterload (amyl nitrate administration), the click and murmur occur earlier in systole. Conversely, with an increase in left ventricular volume (squatting) or increase in afterload (handgrip) the click and murmur occur later.

Other diagnostic factors

common

palpitations

There is no increased incidence of ECG abnormalities.[13][35]

A subset of patients with MVP, particularly those with findings of mitral annular disjunction, are at high arrhythmic risk and continuous ambulatory Holter or event monitoring may be required.[1][32]​​

Risk factors

strong

connective tissue disorders

MVP with characteristic myxomatous degeneration occurs more frequently in certain connective tissue diseases such as Marfan's syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum.[22][23][24]

This suggests that primary MVP may be a related connective tissue disorder.

mitral annular disjunction (MAD)

MAD is a structural abnormality of the mitral annulus fibrosus that results from a separation between the atria- mitral valve junction and the left ventricular (LV) attachment. This results in a gap, that is most noticeable in systole, between the posterior mitral valve leaflet and the basal inferolateral segment. With the use of modern imaging techniques, MAD has been increasingly identified in cases of MVP. MAD is associated with ventricular arrhythmias and sudden death though the understanding of risk stratification of these patients continues to evolve. MAD can occur without MVP and whether the arrhythmic risk is related to MAD itself or MVP remains to be determined.[28]​ Chordal stretch leading to LV and papillary muscle fibrosis is hypothesised to be a substrate for arrhythmogenic complications in these patients.[28][29]​​

weak

family history

In general, mitral valve prolapse (MVP) is a sporadic condition, but familial clustering is well recognised as autosomally dominant with variable penetrance.[10][12]

The familial variant is associated with an increased incidence of sudden cardiac death.[12]

slim body type

This is associated with an increased risk of MVP.

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