Mitral Regurgitation Guidelines

Updated: Nov 28, 2018
  • Author: Ivan Hanson, MD; Chief Editor: Terrence X O'Brien, MD, MS, FACC  more...
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Guidelines

Guidelines Summary

2017 ACC/AHA guidelines

In 2017, the American College of Cardiology TAsk Force on Expert Consensus Pathways published their updated recommendations for mitral regurgitation (MR), which primarily consisted of those for chronic primary and chronic secondary MR and are summarized below. [28]  In general, the 2014 recommendations remain current (see below, under "2014 ACC/AHA guidelines"), with a few updated recommendations.

Chronic primary MR interventions

Class IIa (moderate strength, with limited data)

Mitral valve surgery is reasonable for asymptomatic patients with chronic severe primary MR (stage C1) and preserved left ventricular (LV) function (LV ejection fraction [LVEF] >60% and LV end-systolic dimension [LVESD] < 40 mm) with a progressive increase in LV size or decrease in EF on serial imaging studies.

Chronic secondary MR interventions

Class IIa (moderate strength, moderate quality from randomized trials)

Chordal-sparing MVR is a reasonable selection over downsized annuloplasty repair if the procedure is considered for severely symptomatic patients (New York Heart Association [NYHA] class III to IV) with chronic severe ischemic MR (stage D) and persistent symptoms despite guideline-directed medical therapy (GDMT) for heart failure (HF).

Class IIb (weak, moderate quality from randomized trials)

In patients with chronic, moderate, ischemic MR (stage B) undergoing CABG, the usefulness of mitral valve repair is uncertain.

2017 ESC/EACTS guidelines

The European Society of Cardiology/European Association for Cardio-Thoracic Surgery (ESC/EACTS) published 2017 updated guidelines to their 2014 recommendations for the management of valvular heart disease. [29]

Class I (recommended/indicated; level of evidence that is the consensus opinion of experts and/or small studies, retrospective studies, registries)

Coronary angiography is recommended for evaluating moderate to severe secondary MR.

Class IIa (should be considered; data derived from a single randomized clinical trial or large nonrandomized studies)

Non-vitamin K antagonist oral anticoagulants (NOACs) should be considered as an alternative to vitamin K antagonists (VKAs) in patients with aortic stenosis, aortic regurgitation and MR presenting with atrial fibrillation.

Indications for intervention in severe primary MR

Class I (recommended/indicated)

The preferred technique should be mitral valve repair when the results are expected to last. (Level of evidence: consensus opinion of experts and/or small studies, retrospective studies, registries)

Surgery is indicated in the following individuals (data derived from a single randomized clinical trial or large nonrandomized studies):

  • Symptomatic patients with LVEF over 30%
  • Asymptomatic patients with LV dysfunction (LVESD ≥45 mm and/or LVEF ≤60%)

Class IIa (should be considered)

Surgery should be considered in the following patients:

  • Asymptomatic patients with preserved LV function (LVESD < 45 mm and LVEF >60%) and atrial fibrillation secondary to MR or pulmonary hypertension (systolic pulmonary pressure at rest >50 mmHg) (Data derived from a single randomized clinical trial or large nonrandomized studies.)
  • Asymptomatic patients with preserved LVEF (>60%) and LVESD of 40-44 mm when a durable repair is likely, surgical risk is low, the repair is performed in a heart valve center, and at least one of the following findings is present (consensus opinion of experts and/or small studies, retrospective studies, registries): flail leaflet or presence of significant left atrial (LA) dilatation (volume index ≥60 mL/m 2 BSA) in sinus rhythm

Mitral valve repair should be considered in symptomatic patients with severe LV dysfunction (LVEF < 30% and/or LVESD >55 mm) refractory to medical therapy when the likelihood of successful repair is high and comorbidity low. (Consensus opinion of experts and/or small studies, retrospective studies, registries)

Class IIb (may be considered; consensus opinion of experts and/or small studies, retrospective studies, registries)

Mitral valve replacement may be considered in symptomatic patients with severe LV dysfunction (LVEF < 30% and/or LVESD >55 mm) refractory to medical therapy when the likelihood of successful repair is low and comorbidity low.

Percutaneous edge-to-edge procedure may be considered in patients with symptomatic severe primary MR who fulfil the echocardiographic criteria of eligibility and are judged inoperable or at high surgical risk by the heart team, avoiding futility.

Indications for mitral valve intervention in chronic secondary MR

Class I (should be considered; recommended/indicated; level of evidence that is the consensus opinion of experts and/or small studies, retrospective studies, registries)

Surgery is indicated in patients with severe secondary MR undergoing CABG and an LVEF over 30%.

Class IIa (should be considered; consensus opinion of experts and/or small studies, retrospective studies, registries)

Surgery should be considered in symptomatic patients with severe secondary MR, an LVEF below 30% but with an option for revascularization and evidence of myocardial viability.

Class IIb (may be considered; consensus opinion of experts and/or small studies, retrospective studies, registries)

When revascularization is not indicated, surgery may be considered in patients with severe secondary MR and an LVEF above 30% who remain symptomatic despite optimal medical management (including cardiac resynchronization therapy [CRT] if indicated) and have a low surgical risk.

When revascularization is not indicated and the surgical risk is not low, a percutaneous edge-to-edge procedure may be considered in patients with severe secondary MR and an LVEF over 30% who remain symptomatic despite optimal medical management (including CRT if indicated) and who have a suitable valve morphology by echocardiography, avoiding futility.

In patients with severe secondary MR and an LVEF below 30% who remain symptomatic despite optimal medical management (including CRT if indicated) and who have no option for revascularization, the heart team may consider a percutaneous edge-to-edge procedure or valve surgery after careful evaluation for a ventricular assist device or heart transplant according to each individual patient's characteristics.

2016 AATS guidelines

The 2016 American Association For Thoracic Surgery (AATS) updated recommendations for ischemic mitral valve regurgitation (IMR) are outlined below. [30]

Mitral valve replacement is reasonable in patients with severe IMR who remain symptomatic despite Guideline-directed medial and cardiac device therapy, and who have a basal aneurysm/dyskinesis, significant leaflet tethering, and/or severe left ventricle dilation (left ventricular end diastolic diameter [LVEDD] > 6.5 cm).

Mitral valve repair with an undersized complete rigid annuloplasty ring may be considered in patients with severe IMR who remain symptomatic despite Guideline-directed medical and cardiac device therapy and who do not have a basal aneurysm/dyskinesis, significant leaflet tethering, or severe left ventricle enlargement.

In patients with moderate IMR undergoing CABG, mitral valve repair with an undersized complete rigid annuloplasty ring may be considered.

Mitral valve repair for IMR is performed with complete preservation of both anterior and posterior leaflet chords.

Mitral valve repair for IMR is performed with small, undersized, complete rigid annuloplasty ring.

2014 ACC/AHA guidelines

In 2014, the ACC/American Heart Association (ACC/AHA) released a revision to its 2008 guidelines for the management of patients with valvular heart disease (VHD); and the ESC/EACTS issued a revision of its 2007 guidelines in 2012. [1, 13]

The ACC/AHA guidelines classify progression of chronic mitral regurgitation (MR) into 4 stages (A to D) as follows [13] :

  • Stage A: At risk of MR

  • Stage B: Asymptomatic with progressive MR

  • Stage C: Asymptomatic with severe MR; stage C1 (left [LV] or right ventricle [RV] remains compensated) or stage C2 (decompensation of LV or RV)

  • Stage D: Symptomatic with severe MR

The guidelines note that when assessing chronic MR, it is important to distinguish between chronic primary (degenerative) MR and chronic secondary (functional) MR, as these conditions have more differences than similarities. The staging criteria is summarized in the table below. [13]

Table 1. Stages of Progression of Chronic Mitral Regurgitation (Open Table in a new window)

Stage

Valve Anatomy

Valve Hemodynamics*

Hemodynamic Consequences

Symptoms

A:

Primary MR

  • Mild MV prolapse with normal coaptation
  • Mild valve thickening and leaflet restriction
  • No MR jet or small central jet area < 20% LA
  • Small vena contracta < 0.3 cm
  • None
  • None

A:

Secondary MR

  • Normal valve leaflets, chords and annulus in patient with CAD or cardiomyopathy
  • No MR jet or small central jet area < 20% LA
  • Small vena contracta < 0.3 cm
  • Normal or mildly dilated LV with infarction or ischemia regional wall motion abnormalities
  • Primary myocardial disease with LV dilation and systolic dysfunction
  • Symptoms of coronary ischemia or HF that respond to revascularization and medical therapy

B:

Primary MR

  • Severe MV prolapse with normal coaptation
  • Rheumatic valve changes, leaflet restriction, and loss of central coaptation
  • Prior IE
  • Central jet MR area  20-40% LA or late systolic eccentric jet MR
  • Vena contracts < 0.7 cm
  • Regurgitant volume < 60 mL
  • Regurgitant fraction < 50%
  • ERO < 0.40 cm 2
  • Angiographic grade 1-2+
  • Mild LA enlargement
  • No LV enlargement
  • Normal Pulmonary pressure
  • None

B:

Secondary MR

  • Regional wall motion abnormalities with mild tethering of mitral leaflet
  • Annular dilation with mild loss of central coaptation of the mitral leaflets
  • Regurgitant volume < 30 mL
  • Regurgitant fraction < 50%
  • ERO < 0.20 cm 2†
  • Regional wall motion abnormalities with reduced LV systolic function
  • LV dilation and systolic dysfunction
  • Symptoms of coronary ischemia or HF that respond to revascularization and medical therapy

C:

Primary MR

  • Severe MV prolapse with loss of coaptation or flail leaflet
  • Rheumatic valve changes, leaflet restriction, and loss of central coaptation
  • Prior IE
  • Thickening of leaflets with radiation heart disease
  • Central jet MR area >40% LA or holosystolic eccentric jet MR
  • Vena contracta ≥0.7 cm
  • Regurgitant volume ≥60 mL
  • Regurgitant fraction ≥50%
  • ERO ≥0.40 cm 2
  • Angiographic grade 3-4+
  • Moderate to severe LA enlargement
  • LV enlargement
  • Pulmonary hypertension may be present
  • Stage C1: LVEF >60% and LVESD < 40 mm
  • Stage C2: LVEF ≤60% and LVESD ≥40 mm
  • None

C:

Secondary MR

  • Regional wall motion abnormalities and/or LV dilation with severe tethering of mitral leaflet
  • Annular dilation with severe loss of central coaptation of the mitral leaflets
  • Regurgitant volume ≥30 mL
  • Regurgitant fraction ≥50%
  • ERO ≥0.20 cm 2†
  • Regional wall motion abnormalities with reduced LV systolic function
  • LV dilation and systolic dysfunction
  • Symptoms of coronary ischemia or HF that respond to revascularization and medical therapy

D:

Primary MR

  • Severe MV prolapse with loss of coaptation or flail leaflet
  • Rheumatic valve changes, leaflet restriction, and loss of central coaptation
  • Prior IE
  • Thickening of leaflets with radiation heart disease
  • Central jet MR area >40% LA or holosystolic eccentric jet MR
  • Vena contracta ≥0.7 cm
  • Regurgitant volume ≥60 mL
  • Regurgitant fraction ≥50%
  • ERO ≥0.40 cm 2
  • Angiographic grade 3-4+
  • Moderate or severe LA enlargement
  • LV enlargement
  • Pulmonary hypertension present
  • Decreased exercise tolerance
  • Exertional dyspnea

D:

Secondary MR

  • Regional wall motion abnormalities and/or LV dilation with severe tethering of mitral leaflet
  • Annular dilation with severe loss of central coaptation of the mitral leaflets
  • Regurgitant volume ≥30 mL
  • Regurgitant fraction ≥50%
  • ERO ≥0.20 cm 2†
  • Regional wall motion abnormalities with reduced LV systolic function
  • LV dilation and systolic dysfunction
  • HF symptoms due to MR persist after revascularization and medical therapy
  • Decreased exercise tolerance
  • Exertional dyspnea

*Several criteria are provided but not all criteria for each category will be present. Severity of mild, moderate, or serve is dependent on data quality and integration with other clinical evidence.

In secondary MR, true ERO is underestimated due to the crescentic shape of the proximal convergence

CAD = coronary heart disease; ERO = effective regurgitation orifice; HF = heart failure; IE = infective endocarditis; LA = left atrium; LV = left ventricular; LVEF = left ventricular ejection factor; LVESD = left ventricular end-systolic dimension; MR = mitral regurgitation; MV = mitral valve; TTE = transthoracic echocardiography.

Both guidelines require intervention decisions for severe valvular heart disease (VHD) be based on an individual risk-benefit analysis. Improved prognosis should outweigh the risk of intervention and potential late consequences, particularly complications related to prosthetic valves. [1, 13]

Recognizing the known limitations of the EuroSCORE (European System for  Cardiac Operative Risk Evaluation) and the STS (Society of Thoracic Surgeons) score, the ACC/AHA guidelines suggest using STS plus three additional indicators: frailty (using accepted indices), major organ system compromise not to be improved postoperatively, and procedure-specific impediment when assessing risk. [13]

Diagnosis

The ESC/EACTS echographic criteria for the definition of severe MR are as follows [1] :

  • Flail leaflet/ruptured papillary muscle/large coaptation defect

  • Very large color flow central jet or eccentric jet adhering, swirling, and reaching the posterior wall of the LA

  • Dense/triangular continuous-wave signal of regurgitant jet

  • Large flow convergence zone

ACC/AHA class I indications for transthoracic echocardiography (TTE) are as follows [13] :

  • Baseline evaluation for LV size and function, right ventricular (RV) and LA size, pulmonary artery pressure, and severity of MR

  • Determining the etiology of MR

  • Annual or semiannual surveillance of LV ejection fraction (LVEF) and end-systolic dimension in asymptomatic patients with moderate-to-severe MR

  • Evaluation of the MV apparatus and LV function after a change in signs or symptoms

  • Evaluation of LV size and function and MV hemodynamics in the initial evaluation after MV replacement (MVR) or MV repair

ACC/AHA class I indications for serial TTE are as follows [13] :

  • Asymptomatic patients with mild MR and no evidence of LV enlargement, LV dysfunction, or pulmonary hypertension: Yearly observation; serial TTE is not indicated

  • Patients with moderate MR: Yearly TTE

  • Asymptomatic patients with severe MR: TTE and clinical evaluation every 6-12 months to assess symptoms and development of LV dysfunction

ACC/AHA class I indications for transesophageal echocardiography (TEE) are as follows [13] :

  • Assessment of etiology of severe MR in patients for whom surgery is recommended to determine the feasibility of MV repair

  • Evaluation of MV and associated structures when TTE is nondiagnostic

Acute mitral regurgitation

The ACC/AHA guidelines note that it may be difficult to diagnose severe acute MR with TTE due to narrow eccentric jets of MR, tachycardia, and early equalization of LV and LA pressures. In cases where TTE is nondiagnostic but the suspicion of severe acute MR persists, enhanced MV imaging with TEE is recommended. In patients with sudden acute and hemodynamic instability after myocardial infarction (MI) with hyperdynamic LV function and no other cause for the deterioration, TEE should be performed as soon as possible, looking for severe MR due either to a papillary muscle or chordal rupture. [13]

The guidelines suggest vasodilator therapy to improve hemodynamic compensation in acute MR; however, use of vasodilators is often limited by systemic hypotension that is exacerbated when peripheral resistance is decreased. Intra-aortic balloon counterpulsation can be utilized for achieving hemodynamic stability until definitive mitral surgery can be performed. The use of a percutaneous circulatory assist device may also be effective to stabilize a patient with acute hemodynamic compromise before operation. [13]

Prompt MV surgery is recommended for treatment of the symptomatic patient with acute severe primary MR. In cases of ruptured chordae tendineae, mitral repair is usually feasible and preferred over valve replacement, and the timing of surgery can be determined by the patient’s hemodynamic status. If infectious endocarditis (IE) is the cause of severe symptomatic MR, earlier surgery is generally preferred because of better outcomes over medical therapy. However, this strategy should also be tempered by the patient’s overall condition. [13]

Primary mitral regurgitation

The ACC/AHA and ESC/EACTS guidelines agree that MV surgery for symptomatic patients with chronic severe primary MR (stage D) and LVEF above 30% is a class I recommendation. [1, 13]

The ACC/AHA guidelines also provide a class I recommendation for MV surgery for asymptomatic patients with chronic severe primary MR (stage C2) and an LVEF of 30%–60% and/or an LVESD of at least 40 mm, [13]  whereas the ESC/EACTS uses a benchmark of an LVEF above 30% and an LVESD below 55 mm for its class I recommendation. [1]

The ACC/AHA also gives class I recommendations for MV repair in preference to MVR in the following [13] :

  • Severe primary MR limited to the posterior leaflet

  • Severe primary MR involving the anterior leaflet or both leaflets and a successful and durable repair can be accomplished

The ECS/EACTS guidelines prefer MV repair when a durable repair can be expected. [1]

Additional ACC/AHA recommendations for interventions for primary MR include the following [13] :

  • Concomitant MV repair or replacement for patients undergoing other cardiac surgery (class I for severe primary MR; class IIa for moderate primary MR)

  • MV repair in asymptomatic patients with chronic severe primary MR (stage C1) with LVEF above 60% and LVESD below 40 mm:

    • When the likelihood of a successful and durable repair is over 95% and the expected operational mortality rate is less than 1% and the procedure is performed at a heart valve center of excellence (class IIa)
    • When the likelihood of a successful and durable repair is high and there is new onset of atrial fibrillation (AF) or resting pulmonary hypertension (class IIa)
  • Medical therapy for systolic dysfunction in symptomatic patients with chronic primary MR (stage D) and LVEF below 60% in whom surgery is not contemplated (class IIa)

  • Consider MV surgery in symptomatic patients with chronic severe primary MR and LVEF of 30% or less (class IIb)

  • Consider MV repair in patients with rheumatic MV disease if a durable and successful repair is likely or when reliability of long-term coagulation management is not feasible (class IIb)

  • Consider transcatheter MV repair for severely symptomatic patients (New York Heart Association [NYHA] functional class III-IV) with chronic severe primary MR (stage D) who have favorable anatomy and a reasonable life expectancy but have prohibitive surgical risk due to severe comorbidities and remain severely symptomatic despite optimal GDMT for  HF (class IIb)

  • MVR should not be performed for the treatment of isolated severe primary MR limited to less than one half of the posterior leaflet unless MV repair has been attempted and was unsuccessful (class III).

In general, the ESC/EACTS guidelines are in alignment with the AHA/ACC recommendations. [1]

Secondary mitral regurgitation

The ACC/AHA class I recommendations for management of secondary MR include the following [13] :

  • Patients with chronic secondary MR and HF with reduced LVEF should receive standard medical therapy for HF including angiotensin-converting enzyme (ACE) inhibitors (ACEIs), angiotensin-receptor blockers (ARBs), beta blockers, and/or aldosterone antagonists.

  • Cardiac resynchronization therapy (CRT) with biventricular pacing or symptomatic patients with chronic severe secondary MR who meet the indications for device therapy.

Both the ACC/AHA and ESC/EACTS guidelines recommend MV surgery for patients with chronic severe secondary MR (stages C and D) who are undergoing CABG or AVR (class IIa, ACC/AHA; class I, ESC/EACTS). [1, 13]  The ESC/EACTS guidelines also recommend surgery be considered for patients with moderate MR undergoing CABG (class IIa). [1]

Additional ACC/AHA recommendations include the following [13] :

  • Consider MV repair or replacement for severely symptomatic patients (NYHA class III to IV) with chronic severe secondary MR (stage D) who have persistent symptoms despite optimal medical therapy for HF (class IIb).

  • Consider MV repair for patients with chronic moderate secondary MR (stage B) who are undergoing other cardiac surgery (class IIb).

Infective endocarditis

Both the AHA and ESC released updated guidelines for the management of IE in 2015. [31, 32] Major recommendations from the AHA for the management of IE are summarized below [13, 32] :

Class I

The Modified Duke Criteria should be used in evaluating a patient with suspected IE. (Level of evidence: B)

At least 3 sets of blood cultures from different venipuncture sites should be obtained, with the first and last samples drawn at least 1 hour apart. (Level of evidence: A)

TTE should be performed in all cases of suspected IE. (Level of evidence: B)

TEE should be performed if initial TTE images are negative or inadequate in patients for whom there is an ongoing suspicion for IE or when there is concern for intracardiac complications in patients with an initial positive TTE. (Level of evidence: B)

If there is a high suspicion of IE despite an initial negative TEE, then obtain a repeat TEE in 3 to 5 days or sooner if clinical findings change. (Level of evidence: B)

Repeat TEE should be performed after an initially positive TEE if clinical features suggest a new development of intracardiac complications. (Level of evidence: B)

Before being considered for outpatient therapy, patients with IE should first be evaluated and stabilized in the hospital. (Level of evidence: C)

Appropriate antibiotic therapy should be initiated and continued after blood cultures are obtained, with guidance from antibiotic sensitivity data and infectious disease consultants. (Level of Evidence: B)

Patients selected for outpatient parenteral antibiotic therapy (OPAT) should be at low risk for the complications of IE, the most frequent of which are heart failure and systemic emboli. (Level of evidence: C)

Surgery should be performed before completion of a full therapeutic course of antibiotics in patients with the following: 

  • IE and valve dysfunction resulting in symptoms of HF (Level of evidence: B)
  • IE caused by fungi or highly resistant organisms (eg, vancomycin-resistant  Enterococcus, multidrug-resistant Gram-negative bacilli) (Level of evidence: B)
  • IE complicated by heart block, annular or aortic abscess, or destructive penetrating lesions (Level of evidence: B)
  • Persistent bacteremia or fevers lasting longer than 5 to 7 days after the onset of appropriate antimicrobial therapy (Level of evidence: B)

Valve repair rather than replacement should be performed when feasible. (Level of evidence: C)

Months to years after completion of medical therapy for IE, patients should have ongoing observation for and education about recurrent infection and delayed onset of worsening valve dysfunction. (Level of evidence: C)

Class III

Patients should not receive antibiotics before blood cultures are obtained for unexplained fever. (Level of evidence: C)

Antimicrobial therapy should not be initiated for the treatment of undefined febrile illnesses unless the patient’s condition (eg, sepsis) warrants it. (Level of evidence: C)