Grade
Definition
Valve anatomy
Valve hemodynamicsa
Hemodynamic consequences
Symptoms
A
At risk of MR
• Mild mitral valve prolapse with normal coaptation
• Mild valve thickening and leaflet restriction
• No MR jet or small central jet area <20% LA on Doppler
• Small vena contracta <0.3 cm
• None
• None
B
Progressive MR
• Severe mitral valve prolapse with normal coaptation
• Rheumatic valve changes with leaflet restriction and loss of central coaptation
• Prior IE
• Central jet MR 20–40% LA or late systolic eccentric jet MR
• Vena contracta <0.7 cm
• Regurgitant volume <60 mL
• Regurgitant fraction <50%
• ERO <0.40 cm2
• Angiographic grade 1–2+
• Mild LA enlargement
• No LV enlargement
• Normal pulmonary pressures
• None
C
Asymptomatic severe MR
• Severe mitral valve prolapse with normal coaptation
• Rheumatic valve changes with leaflet restriction and loss of central coaptation
• Prior IE
• Thickening of leaflets with radiation heart disease
• Central jet MR >40% LA or holosystolic eccentric jet MR
• Vena contracta ≥0.7 cm
• Regurgitant volume ≥60 mL
• Regurgitant fraction ≥50%
• ERO ≥0.40 cm2
• Angiographic grade 3–4+
• Moderate or severe LA enlargement
• LV enlargement
• Pulmonary hypertension may be present at rest or with exercise
• C1: LVEF >60% and LVESD <40 mm
• C2: LVEF ≤60% and LVESD ≥40 mm
• None
D
Symptomatic severe MR
• Same as stage C
• Same as stage C
• Moderate or severe LA enlargement
• LV enlargement
• Pulmonary hypertension present
• Decreased exercise tolerance
• Exertional dyspnea
Stages of mitral stenosis
Grade | Definition | Valve anatomy | Valve hemodynamics | Hemodynamic consequences | Symptoms |
---|---|---|---|---|---|
A | At risk of MS | • Mitral valve doming during systole | • Normal transmitral flow velocity | • None | • None |
B | Progressive MS | • Rheumatic valve changes with commissural fusion and diastolic doming of the mitral valve leaflets • Planimetered MVA >1.5 cm2 | • Increased transmitral flow velocities • MVA >1.5 cm2 • Diastolic pressure half-time <150 ms | • Mild-moderate LA enlargement • Normal pulmonary pressure at rest | • None |
C | Asymptomatic severe MS | • Rheumatic valve changes with commissural fusion and diastolic doming of the mitral valve leaflets • Planimetered MVA ≤1.5 cm2 • MVA ≤1.0 cm2 with very severe MS | • MVA ≤1.5 cm2 • MVA ≤1.0 cm2 with very severe MS • Diastolic pressure half-time ≥150 ms • Diastolic pressure half-time ≥220 ms with very severe MS | • Severe LA enlargement • Elevated PASP >30 mmHg | • None |
D | Symptomatic severe MS | • Same as stage C | • Same as stage C | • Same as stage C | • Decreased exercise tolerance • Exertional dyspnea |
Etiology and Classification
Mitral Regurgitation
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Spectrum of degenerative/myxomatous mitral valve disease . From Adams DH, Rosenhek R, Falk V. Degenerative mitral valve regurgitation: best practice revolution. Eur Heart J. 2010;31(16):1958–1966. Reprinted with permission from Oxford University Press. FED fibroelastic deficiency disease
Secondary MR is a disease of the LV that has led to abnormal shape/structure and function that cause displacement of one or both papillary muscles, leaflet tethering and inadequate coaptation, and often annular dilatation [8, 9]. Secondary MR may result from ischemic or nonischemic ventricular disease. Because the mitral valve itself is not the origin of the disease, therapy directed only at MR may reduce regurgitation but cannot cure the basic underlying pathology.
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Carpentier classification of mitral valve regurgitation . Type I exhibits normal leaflet motion but annular dilation or leaflet perforation. Type II is leaflet prolapse. Type III describes leaflet restriction. Type III is further divided into IIIa (restricted opening) and IIIb (restricted closing)
Mitral Stenosis
Mitral stenosis most commonly occurs as a consequence of rheumatic fever, a history of which is noted in approximately 60% of patients with pure MS [3, 5]. Significant annular calcification causing calcific MS is the next most common cause, but relatively infrequently leads to obstruction severe enough to warrant valve replacement.
Diagnosis
Imaging Assessment
Mitral Regurgitation
Two-Dimensional Transthoracic Echocardiography
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Panel a: Transthoracic parasternal long axis view demonstrating the posterior leaflet prolapse. The scallops at the mitral leaflet tips are A2 and P2. Panel b is an apical four chamber view demonstrating the scallops seen in this view are A1, A2, and P1
TTE can provide guidance as to the feasibility of mitral valve repair by providing assessment of scallop anatomy or leaflet tethering and demonstrates the extent of mitral annular calcification. In addition to an assessment of the etiology and anatomy of the MR, TTE provides an accurate assessment of LA and LV size, LV function, and pulmonary artery pressures, all of which are important in clinical management.
Two-Dimensional Transesophageal Echocardiography
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Transesophageal echocardiogram (TEE) demonstrating the different mitral scallops. Panel a: at 0°, A1 and P1 scallops are seen in the high esophageal view with the left ventricular outflow tract in view. Panel b: at 90°, the P3 scallops is seen which appears to prolapse. Panel c: at 120°, A2 and P2 scallops are seen. P2 scallop is flail
Three-Dimensional (3D) Echocardiography: Advantages and Pitfalls over 2D
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3D echocardiogram demonstrating the mitral valve in the “surgeon’s view” with the aortic valve (AoV) at the 12 o’clock location and left atrial appendage (LAA) on the lateral aspect. One can now visualize all six scallops of the mitral valve. Note that the P3 scallop prolapses and is flail
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Transesophageal echocardiogram (TEE) demonstrating that 2D TEE by itself may lead to misrepresentation of scallops. In panel a at 120°, the P2 scallop appears to be flail. However in panel b when 3D echocardiogram is utilized, it is actually the P3 scallop (which is very large) that prolapses rather than the P2 scallop
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3D echocardiography can be utilized to identify mitral clefts, which would not be seen on 2D TEE. Panel a demonstrates 3D of the mitral valve showing indentations between the mitral scallops (red arrows) that extend from the mitral leaflet tips to the annulus. Panel b demonstrates the clefts as seen from the ventricular aspect
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3D echocardiography with color can be used to identify the origin of mitral regurgitation. Shown is an example of P2 prolapse. When 3D with color is used, one can visualize the mitral regurgitation originating from the P2 scallop and directed anteriorly
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“Stitch artifact ” (yellow arrows) can occur in 3D images as a result of an irregular heart rhythm, patient movement or breathing
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When utilizing 3D echocardiography, the echocardiographer has to be able to differentiate echocardiographic drop out (yellow arrow) from true defects
Computed Tomography and Magnetic Resonance Imaging in Assessment of Mitral Regurgitation
Computed tomography (CT) and magnetic resonance imaging (MRI) are infrequently utilized in the evaluation of the mitral valve, but can provide important, complementary information to echocardiography. CT can be utilized for diagnosis of mitral valve prolapse [17]. An advantage of CT over echocardiography is that it not only can help in the diagnosis of mitral valve prolapse, but also can concomitantly assess coronary anatomy [18], LV function [19, 20], and the presence of left atrial appendage thrombus [21]. CT can also be used to evaluate the extent of mitral annular calcification, which may help plan surgery and assess the feasibility of mitral repair. MRI is also useful for the assessment of MR, particularly in patients with poor echocardiographic views. MRI provides an assessment of mitral valve anatomy, quantification of MR severity, and accurate assessment of LV size and function [22].
Left Ventriculography in Assessment of Mitral Regurgitation
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A left ventriculogram demonstrating a hyperdynamic left ventricle with dense opacification of the left atrium in systole consistent with severe mitral regurgitation
Doppler Quantitation of Mitral Regurgitation
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Highly eccentric jets may lead to underestimation of the degree of mitral regurgitation not only by color Doppler but also by the PISA method. In this figure, there is a flail posterior leaflet leading to a highly eccentric jet of severe mitral regurgitation. However, color Doppler appears to underestimate the severity of MR
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Quantification of mitral regurgitation requires identification of the zone of flow convergence. One should identify the narrowest portion of the jet as demonstrated by the yellow arrow (vena contracta). Also, one should identify the PISA radius (white arrow) to allow for calculation of the effective regurgitant orifice area (EROA) using the PISA method. The echocardiographer should also obtain a continuous wave Doppler of the mitral regurgitation since the peak velocity and the velocity time integral are also used to calculate the EROA and the regurgitant volume
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3D echocardiography can be utilized to measure the EROA. One can align orthogonal planes to the regurgitant jet to identify the EROA. The EROA can then be traced. In this figure, the EROA can be visualized en face and reveals an area of 0.24 cm2
Pulsed wave Doppler is another method to qualitatively assess MR severity [24]. In patients with severe MR, the E wave velocity is usually greater than 1.2 m/s; the presence of an A wave dominant mitral inflow pattern virtually excludes the presence of severe MR [27]. Pulsed wave Doppler can also be used to calculate the MR regurgitant volume and fraction using the continuity equation [25]. This method is useful when the MR jet is highly eccentric. However, since the annular measurement is a key component of the analysis, any error in its measurement can produce large errors in the calculation of the regurgitant volume and fraction.
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One of the suggestive signs of severe mitral regurgitation is systolic reversal in pulmonary veins, shown here with flow below the baseline when pulse wave Doppler is placed in the pulmonary vein
Common pitfalls in the echocardiographic assessment of MR severity
• Relying on color Doppler to assess the severity of MR, particularly when the Nyquist limit is too low • Color Doppler is inadequate when jets are highly eccentric • Quantification of MR severity can be limited with an eccentric jet or multiple jets • Underestimating MR severity based on a TEE due to anesthesia and lower blood pressure • EROA may not be a true hemisphere as previously thought • Failure to recognize imaging artifacts (stitch, drop out, etc.) on 3D TEE • Mitral gradients for MS can vary and are highly dependent on heart rate and cardiac output • 2D TEE is limited in the diagnosis of mitral clefts or commissural scallops • Color Doppler to assess MR severity may be deceptive in the setting of acute severe MR |
Echocardiographic Evaluation of Mitral Stenosis
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Differentiating calcific mitral stenosis (MS) from rheumatic MS. Panel a demonstrates a patient with calcific MS. The arrow demonstrates the calcium at the mitral annulus which is classic for calcific MS where the leaflets are generally spared. Panel b demonstrates a patient with rheumatic MS with the calcification most prominent at the leaflet tips. The calcification may also extend into the subvalvular apparatus
Transthoracic Echocardiographic Evaluation of Mitral Stenosis
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Continuous wave Doppler should be utilized to assess mitral gradients. In this example, there was a mean mitral valve gradient of 4 mmHg
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The slope of the E wave can be measured (yellow line) which gives the deceleration time. This can be used to calculate the pressure half-time by multiplying the deceleration time by 0.29. The pressure half-time can then be used to calculate the mitral valve area
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The mitral valve area can be measured via planimetered in the parasternal short axis view
Transesophageal Echocardiography in Assessment of Mitral Stenosis
TEE is usually employed prior to balloon valvuloplasty to confirm the absence of an LA appendage thrombus, assess the degree of concomitant mitral regurgitation, better assess the degree of leaflet and subvalvular calcification and thickening [31], and develop the Wilkins score for predicting the outcome of percutaneous balloon mitral valvuloplasty [32]. In this evaluation, one to four points each is given according to ascending severity for leaflet mobility, leaflet calcification, leaflet thickening and subvalvular disease yielding a score ranging from 4–16. A score of 8 or less is predicts a suitable percutaneous valvuloplasty. In contrast, a valve with a score of 12 or more portends an unfavorable outcome. Contraindications for percutaneous balloon mitral valvuloplasty include the presence of moderate or more mitral regurgitation and the presence of a left atrial appendage thrombus. 3D TEE can also be used to assess the mitral orifice area and is more accurate than 2D planimetric measurements.
Strengths and limitations of various echocardiographic modalities for assessing mitral regurgitation and stenosis
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