Approach

Patients without symptoms generally require no therapy. Increased risk of developing severe mitral regurgitation (MR) is associated with male sex, age greater than 45 years, elevated BMI, hypertension, left ventricular (LV) enlargement, LV dysfunction, and thickened mitral valve leaflets.[5][6][38][39][40]​​ MVP-related MR is typically managed similarly to other forms of MR, but surgical intervention may be more attractive for those with severe MR related to MVP because of the greater likelihood of successful repair.

Asymptomatic patients

Most asymptomatic patients with MVP need no specific treatment and should be reassured of their excellent prognosis. Normal lifestyle and regular exercise should be encouraged. Patients who have a dilated left ventricle, left atrial dilation, pulmonary hypertension, and new atrial fibrillation can be considered for surgery in combination with other clinical factors.

Palpitations, anxiety, atypical chest pain

Palpitations and atypical symptoms warrant investigation, but if the evaluation has been unrevealing, a trial of lifestyle changes, including avoidance of stimulants and alcohol, may be effective, especially for individuals with increased adrenergic responses to activity, stress, or stimulants. Empiric beta-blocker treatment may be useful in alleviating symptoms of palpitations, anxiety, or atypical chest pain in certain patients.[41]

The approach should be adjusted for patients with mitral annular disjunction (MAD). Patients with MAD have a slightly higher rate of arrhythmias and should undergo cardiac monitoring if they experience any palpitations. For those with arrhythmic MVP but no history of severe events, the yearly incidence of significant ventricular arrhythmias, such as ventricular fibrillation, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator therapy, can reach up to 4%.[26]

Embolization risk

There is disagreement as to whether MVP can cause strokes. The proposed mechanism is embolization of platelet-fibrin thrombi from the leaflet surface. Patients with MVP who have strokes tend to have risk factors such as older age (>50 years), severe MR, atrial fibrillation, and thickened valve leaflets.[6][42]​ Data suggest a yearly event rate of 0.7%.[43]

MVP should rarely be considered the sole source of stroke. Depending on the presence of further risk factors, aspirin, warfarin, or a direct oral anticoagulant is recommended.[1][44][45][46]

Mitral regurgitation

Mild MR

  • MR is common in MVP. Most patients are asymptomatic, and no specific therapy is required. Atrial fibrillation or indicators of hemodynamic compromise, such as LV dysfunction or pulmonary hypertension, are more typically associated with severe MR in MVP patients.

Severe primary MR

  • Patients with severe primary MR may warrant early consideration for surgical intervention, even when asymptomatic. For asymptomatic patients, surgery is indicated for LV dysfunction (ejection fraction ≤60%, LV end-systolic diameter ≥40 mm).[1][2][45]​ Intervention for asymptomatic patients with severe primary MR is also considered for those with elevated pulmonary artery pressure, atrial fibrillation, or when there is significant left atrial dilatation and when there is a high likelihood of successful surgical repair and a low surgical risk.[1][45]

  • There is some controversy over the management of patients with MVP and severe primary MR who are asymptomatic without evidence of LV dysfunction, pulmonary hypertension, or atrial fibrillation. American College of Cardiology/American Heart Association (ACC/AHA) guidelines have recommended mitral valve surgery as reasonable for this group, in experienced surgical centers, in cases where the likelihood of repair is >95% with an estimated surgical mortality of ≤1%, or if there is a progressive increase in LV size or progressive decrease in EF on ≥3 serial imaging studies.[1][2] The European Society of Cardiology (ESC) recommends watchful waiting in asymptomatic patients with severe primary mitral regurgitation and no indications for surgery.[45]

  • ACC/AHA and ESC guidelines recommend surgery for all symptomatic patients with severe chronic primary MR and acceptable surgical risk.[1][2][45] For symptomatic patients who are inoperable or at high surgical risk, transcatheter mitral valve intervention (transcatheter edge-to-edge repair) may be considered if mitral valve anatomy is favorable.[1][2][45]

  • Mitral valve repair is technically possible in most instances in prolapse or flail of the posterior leaflet but is more difficult in anterior leaflet prolapse, flail anterior leaflet, or bileaflet prolapse. The advantages of repair over replacement include lower operative and long-term mortality, better preservation of LV systolic function, and avoidance of the need for anticoagulation associated with a mechanical prosthetic valve. Patients undergoing mitral valve repair are usually treated in the postoperative period with aspirin and require anticoagulation only if atrial fibrillation is present.[1][44][45]

  • When surgery is indicated but is not possible or must be delayed, ACC/AHA guidelines recommend that guideline-directed medical therapy for systolic dysfunction may be considered.[1] ESC guidelines recommend medical treatment in line with current heart failure guidelines for patients with overt heart failure.[45] Medical therapy with afterload reduction has not been shown to impact outcomes or postpone the need for surgery in patients with severe primary MR due to MVP and normal LV function, and is contraindicated. 

  • Mitral valve repair is the preferred treatment option in patients with MVP and severe primary MR requiring surgery. Transcatheter mitral valve intervention has been shown to lead to a reduction in the severity of MR and an improvement in LV volumes. Transcatheter intervention with a clip device is indicated in patients with severe primary MR who have failed appropriate medical therapy for heart failure, and who are not considered surgical candidates because of prohibitive risk, yet are expected to have a life expectancy of >1 year. Clip procedures are most likely to be successful when prolapse or flail segments leading to MR are centrally located, such as segments A2 or P2.

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