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]Marks AR, Choong CY, Sanfilippo AJ, et al. Identification of high-risk and low-risk subgroups of patients with mitral-valve prolapse. N Engl J Med. 1989 Apr 20;320(16):1031-6.
http://www.ncbi.nlm.nih.gov/pubmed/2927482?tool=bestpractice.com
[6]Nishimura RA, McGoon MD, Shub C, et al. Echocardiographically documented mitral valve prolapse: long-term follow-up of 237 patients. N Engl J Med. 1985 Nov 21;313(21):1305-9.
http://www.ncbi.nlm.nih.gov/pubmed/4058522?tool=bestpractice.com
[38]Olson LJ, Subramanian R, Ackermann DM, et al. Surgical pathology of the mitral valve: a study of 712 cases spanning 21 years. Mayo Clin Proc. 1987 Jan;62(1):22-34.
http://www.ncbi.nlm.nih.gov/pubmed/3796056?tool=bestpractice.com
[39]Wilcken DE, Hickey AJ. Lifetime risk for patients with mitral valve prolapse of developing severe valve regurgitation requiring surgery. Circulation. 1988 Jul;78(1):10-4.
http://www.ncbi.nlm.nih.gov/pubmed/3383395?tool=bestpractice.com
[40]Hayek, E, Gring GN, Griffin BP. Mitral valve prolapse. Lancet. 2005 Feb 5-11;365(9458):507-18.
http://www.ncbi.nlm.nih.gov/pubmed/15705461?tool=bestpractice.com
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]Winkle RA, Lopes MG, Goodman DJ, et al. Propranolol for patients with mitral valve prolapse. Am Heart J. 1977 Apr;93(4):422-7.
http://www.ncbi.nlm.nih.gov/pubmed/842437?tool=bestpractice.com
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]Van der Bijl P, Stassen J, Haugaa KH, et al. Mitral annular disjunction in the context of mitral valve prolapse: identifying the at-risk patient. JACC Cardiovasc Imaging. 2024 Apr 18:S1936-878X(24)00119-0.
https://www.sciencedirect.com/science/article/pii/S1936878X24001190?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/38703174?tool=bestpractice.com
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]Nishimura RA, McGoon MD, Shub C, et al. Echocardiographically documented mitral valve prolapse: long-term follow-up of 237 patients. N Engl J Med. 1985 Nov 21;313(21):1305-9.
http://www.ncbi.nlm.nih.gov/pubmed/4058522?tool=bestpractice.com
[42]Orencia AJ, Petty GW, Khandheria BK, et al. Risk of stroke with mitral valve prolapse in population-based cohort study. Stroke. 1995 Jan;26(1):7-13.
http://stroke.ahajournals.org/cgi/content/full/26/1/7
http://www.ncbi.nlm.nih.gov/pubmed/7839400?tool=bestpractice.com
Data suggest a yearly event rate of 0.7%.[43]Avierinos JF, Brown RD, Foley DA, et al. Cerebral ischemic events after diagnosis of mitral valve prolapse: a community-based study of incidence and predictive factors. Stroke. 2003 Jun;34(6):1339-44.
http://stroke.ahajournals.org/cgi/content/full/34/6/1339
http://www.ncbi.nlm.nih.gov/pubmed/12738894?tool=bestpractice.com
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]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
[44]Kleindorfer DO, Towfighi A, Chaturvedi S, et al. 2021 Guideline for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline from the American Heart Association/American Stroke Association. Stroke. 2021 Jul;52(7):e364-e467.
https://www.doi.org/10.1161/STR.0000000000000375
http://www.ncbi.nlm.nih.gov/pubmed/34024117?tool=bestpractice.com
[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
[46]Lansberg MG, O'Donnell MJ, Khatri P, et al. Antithrombotic and thrombolytic therapy for ischemic stroke: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest. 2012 Feb;141(2 Suppl):e601S-36S.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3278065
http://www.ncbi.nlm.nih.gov/pubmed/22315273?tool=bestpractice.com
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]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
[2]Bonow RO, O'Gara PT, Adams DH, et al. 2020 Focused update of the 2017 ACC expert consensus decision pathway on the management of mitral regurgitation: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2020 May 5;75(17):2236-70.
https://www.doi.org/10.1016/j.jacc.2020.02.005
http://www.ncbi.nlm.nih.gov/pubmed/32068084?tool=bestpractice.com
[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
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]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
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]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
[2]Bonow RO, O'Gara PT, Adams DH, et al. 2020 Focused update of the 2017 ACC expert consensus decision pathway on the management of mitral regurgitation: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2020 May 5;75(17):2236-70.
https://www.doi.org/10.1016/j.jacc.2020.02.005
http://www.ncbi.nlm.nih.gov/pubmed/32068084?tool=bestpractice.com
The European Society of Cardiology (ESC) recommends watchful waiting in asymptomatic patients with severe primary mitral regurgitation and no indications for surgery.[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
ACC/AHA and ESC guidelines recommend surgery for all symptomatic patients with severe chronic primary MR and acceptable surgical risk.[1]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
[2]Bonow RO, O'Gara PT, Adams DH, et al. 2020 Focused update of the 2017 ACC expert consensus decision pathway on the management of mitral regurgitation: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2020 May 5;75(17):2236-70.
https://www.doi.org/10.1016/j.jacc.2020.02.005
http://www.ncbi.nlm.nih.gov/pubmed/32068084?tool=bestpractice.com
[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
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]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
[2]Bonow RO, O'Gara PT, Adams DH, et al. 2020 Focused update of the 2017 ACC expert consensus decision pathway on the management of mitral regurgitation: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2020 May 5;75(17):2236-70.
https://www.doi.org/10.1016/j.jacc.2020.02.005
http://www.ncbi.nlm.nih.gov/pubmed/32068084?tool=bestpractice.com
[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
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]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
[44]Kleindorfer DO, Towfighi A, Chaturvedi S, et al. 2021 Guideline for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline from the American Heart Association/American Stroke Association. Stroke. 2021 Jul;52(7):e364-e467.
https://www.doi.org/10.1161/STR.0000000000000375
http://www.ncbi.nlm.nih.gov/pubmed/34024117?tool=bestpractice.com
[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
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]Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e72-e227.
https://www.doi.org/10.1161/CIR.0000000000000923
http://www.ncbi.nlm.nih.gov/pubmed/33332150?tool=bestpractice.com
ESC guidelines recommend medical treatment in line with current heart failure guidelines for patients with overt heart failure.[45]Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28:ehab395.
https://academic.oup.com/eurheartj/article/43/7/561/6358470
http://www.ncbi.nlm.nih.gov/pubmed/34453165?tool=bestpractice.com
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.