Also known as Barlow's syndrome or floppy mitral valve
Prolapsing (billowing) mitral valve
Due to excessively large mitral valve leaflets
An enlarged mitral annulus
Abnormally long chordae
Disordered papillary muscle contraction
Myxomatous degeneration
Histological finding in prolapsing mitral valve
Prolapsing (billowing) mitral valve is more commonly seen in young women than in men or older women
Prolapsing (billowing) mitral valve has a familial incidence
Prolapsing (billowing) mitral valve
Associated with Marfan's syndrome, thyrotoxicosis, rheumatic or ischaemic heart disease
Prolapsing (billowing) mitral valve
Occurs in association with atrial septal defect and as part of hypertrophic cardiomyopathy
Mild mitral valve prolapse is so common that it should be regarded as a normal variant
Mitral valve prolapse (MVP)
Also variously termed the systolic click-murmur syndrome, Barlow's syndrome, floppy-valve syndrome, and billowing mitral leaflet syndrome
MVP
A relatively common but highly variable clinical syndrome
Results from diverse pathogenic mechanisms of the mitral valve apparatus
Excessive or redundant mitral leaflet tissue
Commonly associated with myxomatous degeneration and greatly increased concentrations of acid mucopolysaccharide
Cause of MVP
In most patients, unknown
In some, a genetically determined collagen disorder with a reduction in the production of type III collagen
MVP
A frequent finding in patients with heritable disorders of connective tissue, including Marfan syndrome, osteogenesis imperfecta, and Ehler-Danlos syndrome
MVP
May be associated with thoracic skeletal deformities similar to but not as severe as those in Marfan syndrome, encompassing a high-arched palate and alterations of the chest and thoracic spine, including the so-called straight back syndrome
MVP
In most patients, myxomatous degeneration is confined to the mitral (or, less commonly, the tricuspid or aortic) valves without other clinical or pathologic manifestations of disease
The posterior leaflet is usually more affected than the anterior, and the mitral valve annulus is often greatly dilated
Elongated, redundant, or ruptured chordae tendineae cause or contribute to the regurgitation
MVP
May occur rarely as a sequel to acute rheumatic fever, in ischemic heart disease, and in various cardiomyopathies, as well as in 20% of patients with ostium secundum atrial septal defect
MVP
May lead to excessive stress on the papillary muscles, which in turn leads to dysfunction and ischemia of the papillary muscles and the subjacent ventricular myocardium
Rupture of chordae tendineae and progressive annular dilatation and calcification also contribute to valvular regurgitation, which then places more stress on the diseased mitral valve apparatus, thereby creating a vicious circle
The ECG changes and ventricular arrhythmias appear to result from regional ventricular dysfunction related to increased stress placed on the papillary muscles
Mitral valve prolapse (MVP)
More common in females
Occurs most commonly between ages 15 and 30 years
Clinical course is often benign
May also be observed in older (>50 years) patients, often males, in whom MR is often more severe and requires surgical treatment
Increased familial incidence, suggesting an autosomal dominant form of inheritance
Encompasses a broad spectrum of severities, ranging from only a systolic click and murmur and mild prolapse of the posterior leaflet of the mitral valve to severe MR due to chordal rupture and massive prolapse of both leaflets
Condition may progress over years or decades, or worsen rapidly as a result of chordal rupture or endocarditis
Asymptomatic
Most patients are asymptomatic and remain so for their entire lives
MVP is now the most common cause of isolated severe MR requiring surgical treatment in North America
Arrhythmias associated with MVP
Ventricular premature contractions
Paroxysmal supraventricular tachycardia
Paroxysmal ventricular tachycardia
Atrial fibrillation
Arrhythmias
May cause palpitations, light-headedness, and syncope
Sudden death
Very rare complication, occurs most often in patients with severe MR and depressed LV systolic function
May have an excess risk in patients with a flail leaflet
Chest pain
Often substernal, prolonged, and poorly related to exertion, and it rarely resembles angina pectoris
Transient cerebral ischemic attacks
May be secondary to emboli from the mitral valve due to endothelial disruption, though a causal relationship has not been established
Infective endocarditis may occur in patients with MR and/or leaflet thickening
Auscultation
The most important finding is the mid- or late (non-ejection) systolic click, which occurs 0.14 s or more after the S1 and is thought to be generated by the sudden tensing of slack, elongated chordae tendineae or by the prolapsing mitral leaflet when it reaches its maximum excursion
Systolic clicks
May be multiple and may be followed by a high-pitched, late systolic crescendo-decrescendo murmur, which occasionally is "whooping" or "honking" and is heard best at the apex
Occur earlier with standing, during the strain of the Valsalva maneuver, and with any intervention that decreases LV volume, exaggerating the propensity of mitral leaflet prolapse
Diminish with squatting and isometric exercises, which increase LV volume, diminish MVP, and the click-murmur complex is delayed, moves away from S1, and may even disappear
Some patients have a mid-systolic click without the murmur; others have the murmur without a click. Still others have both sounds at different times
ECG
Most commonly is normal but may show biphasic or inverted T waves in leads II, III, and aVF, and occasionally supraventricular or ventricular premature beats
TTE
Particularly effective in identifying the abnormal position and prolapse of the mitral valve leaflets
A useful echocardiographic definition of MVP is systolic displacement (in the parasternal long axis view) of the mitral valve leaflets by at least 2 mm into the LA superior to the plane of the mitral annulus
Color flow and continuous wave Doppler imaging
Helpful in revealing and evaluating associated MR
TEE
Indicated when more accurate information is required and is performed routinely for intraoperative guidance for valve repair
Invasive left ventriculography
Rarely necessary but can also show prolapse of the posterior and sometimes of both mitral valve leaflets
Infective endocarditis prophylaxis
Indicated only for patients with a prior history of endocarditis
Beta blockers
Sometimes relieve chest pain and control palpitations
Mitral valve repair (or rarely, replacement)
Indicated if the patient is symptomatic from severe MR
Antiplatelet agents such as aspirin
Should be given to patients with transient ischemic attacks
Anticoagulants such as warfarin
Should be considered if antiplatelet agents are not effective