Mitral Valve Prolapse

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Practice Essentials

Mitral valve prolapse (MVP) is the most common valvular abnormality, affecting approximately 2-3% of the population in the United States.[1] MVP usually has a benign course, but it occasionally leads to serious complications, including clinically significant mitral regurgitation (MR), infective endocarditis, sudden cardiac death, and cerebrovascular ischemic events.[2]

Signs and Symptoms

Most patients with MVP are asymptomatic. Symptoms are related to one of the following:

Symptoms related to the progression of MR include the following:

Symptoms related to autonomic dysfunction are usually associated with genetically inherited MVP and may include the following:

Common general physical features associated with MVP include the following:

The classic auscultatory finding is a mid-to-late systolic click. It may or may not be followed by a high-pitched, mid-to-late systolic murmur at the cardiac apex. These can vary with the following maneuvers:

See Presentation for more detail.

Diagnosis

Findings on echocardiography are as follows:

Other echocardiographic findings that should be considered as criteria are leaflet thickening, redundancy, annular dilatation, and chordal elongation.

See Workup for more detail.

Management

For purposes of treatment, patients with MVP can be divided into the following categories:

Treatment measures for asymptomatic patients with minimal disease

Treatment measures for patients with symptoms of autonomic dysfunction

Treatment measures for patients with evidence of or progression to severe MR

Treatment measures for patients with central neurologic findings

See Treatment and Medication for more detail.

Background

Mitral valve prolapse (MVP) is the most common valvular abnormality, affecting approximately 2-3% of the population in the United States.[3, 4] It is generally defined as the systolic displacement of one or more abnormally thickened, redundant mitral leaflet(s) into the left atrium during systole.[5] It may be familial or sporadic, with the majority of data favoring an autosomal dominant inheritance pattern in a large proportion of individuals with MVP.[6, 7] It is also the most common cause of isolated mitral regurgitation (MR) requiring surgical repair.[8]

MVP usually results in a benign course. However, it occasionally leads to serious complications, including clinically significant MR, infective endocarditis, sudden cardiac death, and cerebrovascular ischemic events.

Pathophysiology

Mitral valve prolapse (MVP) is characterized primarily by myxomatous degeneration of the mitral valve leaflets. In younger populations, there is gross redundancy of both the anterior and posterior leaflets and chordal apparatus. This is the extreme form of myxomatous degeneration, known as Barlow syndrome. In older populations, however, MVP is characterized by fibroelastic deficiency, sometimes with superimposed chordal rupture due to a lack of connective tissue support. These anatomic abnormalities result in malcoaptation of mitral valve leaflets during systole, resulting in regurgitation. Mitral annular dilatation may also develop over time, resulting in further progression of mitral regurgitation (MR). Acute severe MR results in congestive heart failure symptoms without left ventricular dilatation. Conversely, chronic or progressively severe MR can lead to ventricular dilatation and dysfunction, neurohormonal activation, and heart failure. Elevation in left atrial pressures can result in left atrial enlargement, atrial fibrillation, pulmonary congestion, and pulmonary hypertension.[9]

Myxomatous proliferation is the most common pathologic basis for MVP, and it can lead to myxomatous degeneration of the loose spongiosa and fragmentation of the collagen fibrils. Disruption of the endothelium may predispose patients to infectious endocarditis and thromboembolic complications. However, most patients with MVP have only a minor derangement of the mitral valve structure that is usually clinically insignificant.

Etiology

Mitral valve prolapse (MVP) usually occurs as an isolated entity. It also commonly occurs with heritable disorders of connective tissue, including Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum.[1, 10] MVP has also been described in association with a secundum atrial septal defect and hypertrophic cardiomyopathy. (Also see Presentation.)

Epidemiology

United States data

Mitral valve prolapse (MVP) is thought to be inherited with increased gene expression in female individuals (2:1). The most common form of inheritance is autosomal dominant, but X-linked inheritance has been described.[11]

MVP commonly occurs with heritable connective tissue disorders,[2] including Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum. In fact, 75% of patients with Marfan syndrome have MVP due to the increased redundancy of the mitral leaflets and apparatus that occurs due to myxomatous degeneration.

MVP was overdiagnosed in the 1970s and 1980s because of the absence of rigorous echocardiographic criteria, with a reported prevalence of 5-15%. Subsequently, Levine et al reported that the two-dimensional echocardiographic characterizations of prolapse, especially on the parasternal long-axis view, are most specific for diagnosing MVP. The use of these criteria prevents overdiagnosis.

Data from the community-based Framingham Heart Study demonstrated that MVP syndrome occurred in only 2.4% of the population.

Age- and sex-related demographics

MVP has been observed in individuals of all ages.

The prevalence of MVP was similar for men and women in the Framingham Heart Study.[3] However, in the Olmsted County population, women were diagnosed more often than men and at a younger age.[12] Complications related to MVP, however, are reported more frequently in men.[13] In a study from the Mayo Clinic, women presented more commonly with anterior and bileaflet prolapse, fewer flail leaflets, and less mitral regurgitation than men.[14] They also undergo mitral surgery less often than men and exhibit excess long-term mortality but equivalent survival after valve surgery compared to men.[14]

Prognosis

Mitral valve prolapse (MVP) has a widely varying prognostic spectrum, although most patients with MVP remain asymptomatic and have a near-normal life expectancy. Approximately 5-10% of patients with MVP progress to severe mitral regurgitation (MR).[15, 16] Overall, young patients (age < 50 years) with normal left ventricular function and no symptoms tend to have excellent survival with medical treatment, even those with severe MR.[12, 17]

Morbidity/mortality

Most patients with MVP are asymptomatic and have a benign prognosis, with survival rates similar to those of the general population. Nonetheless, high-risk patients (ie, those with moderate-to-severe MR and reduced left ventricular systolic function) have increased cardiac morbidity and mortality rates.

In a community-based study of 833 individuals diagnosed with asymptomatic MVP who had longitudinal follow-up in Olmsted County, Minnesota, the best predictors for cardiac mortality were the presence of MR and left ventricular dysfunction at the time of diagnosis.[18] The study also identified certain risk factors for cardiac morbidity (defined as the occurrence of heart failure, thromboembolic events, atrial fibrillation, or need for cardiac surgery), including age older than 50 years, left atrial enlargement, the degree of regurgitation, the presence of a flail leaflet, and the presence of atrial fibrillation at the time of the baseline echocardiogram.[18]

Complications

Complications of mitral valve prolapse include the following:

Mitral regurgitation (MR)

MR is the most common complication of MVP and the leading cause of isolated MR requiring mitral valve surgery in the United States. Acute severe MR is most frequently due to rupture of the chordae tendineae.

The risk of MR increases with the following factors: age older than 50 years, male sex, history of hypertension, increased body mass index (BMI), increased mitral valve thickness or redundancy, and left atrial and left ventricular dilatation.

In a Mayo clinic series, MR volume increased by more than 8 mL in 51% of 74 patients with MVP over a follow-up period of 1.5 years.[19] Progression of the valvular lesions and an increase in mitral annular diameter were the two independent predictors of regurgitant volume over time.[19] MR diagnosed on the basis of the presence of a systolic murmur is associated with an increased risk of adverse events, including progressive valve dysfunction,[20, 21, 22] infective endocarditis,[20, 23] and sudden death.[24, 25]

Infective endocarditis

The main mechanism for an increased risk of infective endocarditis is a turbulent flow state due to leaflet thickness or redundancy and MR. The risk of endocarditis increases 3- to 8-fold with MVP. The risk of developing infective endocarditis is higher if a systolic murmur of MR is present.[26]

The main predictors of infective endocarditis are age older than 50, male sex, history of hypertension, increased BMI, left atrial and ventricular enlargement, and increased mitral valve thickness or redundancy.

In a systematic review of 287 patients undergoing surgery for native valve endocarditis at the Mayo Clinic, MVP was present in 43% of patients and was an independent risk factor for the development of endocarditis.[27]

Cerebrovascular ischemic events

There is only a weak relationship between MVP and stroke. In a study of 777 patients with MVP who were followed for a mean period of 5.5 years, ischemic neurologic events occurred in 4% (n = 30).[28] The risk was significantly increased compared to the expected rate in the community. However, the risk was nondetectable in patients with MVP without leaflet thickening but high after either atrial fibrillation or cardiac surgery.[28] Other studies on MVP have found no relationship between MVP and cerebral events.[3] Gilon et al described the lack of an association between MVP and stroke in 213 young patients.[29] ​

The potential mechanisms for stroke in patients with MVP are related to the prevalence of other risk factors, primarily atrial fibrillation, which may be paroxysmal or asymptomatic. Other hypothesized mechanisms include the formation of platelet fibrin thrombi on the denuded, damaged myxomatous valves resulting in embolization. Data suggest that the prevalence of this mechanism is based on the degree of MR. Abnormal platelet function has also been reported in some studies,[30] although this has been questioned as platelet aggregates on pathology are uncommon.

The major risk factors for cerebrovascular events include age older than 50 years, thickened mitral valve leaflets, atrial fibrillation, and a need for mitral valve surgery.

Sudden cardiac death (SCD) and arrhythmias

There is a 50-60% increased risk of atrial and ventricular arrhythmias in patients with myxomatous MVP; the risk of sudden death is 0.4-2% in these patients.[31, 32, 33] The SCD risk is increased when patients have evidence of left ventricular dilatation and dysfunction, severe MR, or increased mitral leaflet thickness or redundancy.

A clinical syndrome of bileaflet MVP syndrome has been described as a clinical syndrome characterized by myxomatous bileaflet MVP, T-wave inversion in the inferolateral electrocardiograph (ECG) leads, and complex ventricular ectopy (arising from one or both of the papillary muscles), most often observed in young women.[34] In a registry study of 650 adults younger than 40 years with SCD, 43 patients were identified with MVP, comprising 7% of all patients with SCD.[35] Bileaflet involvement was seen in 70%. All patients had left ventricular fibrosis at the level of the papillary muscle on histology, and 88% had inferobasal fibrosis. ECGs for 12 of those patients showed a right bundle-branch block.[35] Factors cited as increasing the risk for life-threatening ventricular arrhythmias include bileaflet MVP, female sex, and frequent ventricular ectopy.[34]

In the presence of QT prolongation and frequent ventricular ectopy, especially nonsustained ventricular or sustained ventricular tachycardia, an electrophysiologic study may be indicated to quantitate the risk of inducible ventricular tachycardia and/or ventricular fibrillation and sudden arrhythmic death.

Patient Education

Patients with mitral valve prolapse (MVP) should receive education regarding their prognosis and the signs and symptoms of disease progression.

Of emphasis, most patients with MVP have a benign course. Still, the risk of ruptured chordae tendinae and/or clinically significant mitral regurgitation, infective endocarditis, embolic transient ischemic attack or stroke, and rare sudden death must also be discussed.

Clinical Classification

Mitral valve prolapse (MVP) can be categorized into primary and secondary MVP. The etiology of primary MVP is abnormal mitral valve leaflet tissue, whereas secondary MVP has an associated disorder, most commonly a connective tissue condition.

Primary MVP

In the community-based Framingham Heart Study, the prevalence of MVP was 2.4%.[3] A similar prevalence was described in a population-based sample of American Indians[4] as well as in a sample of Canadians of South Asian, European, and Chinese descent (SHARE study).[30] Tricuspid valve prolapse has been observed in up to 40-50% of patients with primary MVP.[36]

Primary MVP can occur sporadically or be familial (most commonly inherited in an autosomal dominant pattern with variable penetrance).

Primary MVP is further classified as nonsyndromic or syndromic on the basis of the absence or presence of extracardiac manifestations.

Younger patients commonly present with myxomatous changes and redundancy of both mitral valve leaflets, known as Barlow disease.

Secondary MVP

MVP also occurs in the presence of connective tissue disorders, such as the following[1, 10, 11] :

Secondary MVP has been noted to occur in the setting of congenital heart disease, particularly in Ebstein anomaly, affecting up to 15% of patients.[37]

MVP or a flail leaflet can be caused by non-myxomatous conditions, including myocardial ischemia leading to a ruptured papillary muscle, chest wall trauma, and rheumatic fever.

History

Mitral valve prolapse (MVP) is often diagnosed from the physical examination when the classic auscultatory finding of a mid-to-late systolic click followed by a high-pitched systolic murmur is heard.[11] Alternatively, it may be incidentally diagnosed during routine echocardiography ordered for a different indication or discovered when complications of MVP manifest.

Most patients are asymptomatic. Those with symptomatic MVP typically present with symptoms related to (1) progression of mitral regurgitation (MR), (2) an associated complication (ie, stroke, endocarditis, or arrhythmia), or (3) autonomic dysfunction (this association remains unconfirmed; see below).

Progression of MR

Symptoms related to the progression of MR include the following:

Autonomic dysfunction

Autonomic dysfunction has been reported as the mechanism underlying symptoms in patients with MVP. The spectrum of symptoms resulting from autonomic dysfunction was previously termed MVP syndrome.[3, 38] However, the association between MVP and autonomic dysfunction remains unconfirmed.[39]

Symptoms related to autonomic dysfunction are usually associated with genetically inherited MVP and may include the following:

The electrocardiogram (ECG) is usually normal. Still, it can show nonspecific ST-segment and T-wave abnormalities, especially in the inferior leads and, although uncommon, QT-interval prolongation has been observed in patients with MVP.

As noted earlier, MVP is commonly seen in patients with heritable connective tissue disorders.

Physical Examination

Clinical characteristics of mitral valve prolapse (MVP) are typically benign in young women, whereas men older than 50 years tend to develop more serious consequences of mitral regurgitation (MR).

Common general physical features associated with MVP include the following:

The above features are more commonly seen in patients with secondary MVP causes and less commonly in patients with idiopathic MVP.

Physical findings

The classic auscultatory finding is a mid-to-late systolic click, which is present due to the leaflets prolapsing into the left atrium, resulting in tensing of the mitral valve apparatus. The click may or may not be followed by a high-pitched, mid-to-late systolic murmur at the cardiac apex.

The midsystolic click can vary in intensity and timing, primarily depending on the left ventricular volume.

End-diastolic volume can be reduced by having the patient perform a Valsalva maneuver or by having the patient stand. These maneuvers result in an earlier click, closer to the first heart sound, and a prolonged murmur. They may even bring out a murmur when none is heard at rest. In the supine position, especially with the legs raised for increased venous return, left ventricular diastolic volume increases, resulting in a click later in systole and a shortened murmur. Maneuvers such as squatting or hand grip will also increase end-diastolic left ventricular volume, shifting the click to later in systole.

Echocardiography

Two-dimensional (2D) transthoracic echocardiography (TTE)

TTE is the most important study for diagnosing mitral valve prolapse (MVP).

Perloff et al set the stage for accurately diagnosing MVP by expanding the diagnostic standards to include clinical and echocardiographic criteria.[41, 42]

In a Framingham Heart Study, Freed et al historically described echocardiographic criteria for MVP as classic versus nonclassic (see below).[3]

Use of the parasternal long-axis view reduces the overdiagnosis of MVP.[43]

Echocardiographic findings/diagnostic criteria are as follows:

Thickness of the mitral valve leaflet is measured at the thickest midportion of the leaflet in diastole, from the leading edge to the trailing edge.[44]

2D and 3D transesophageal echocardiography (TEE)

2D-TEE considers several planes of imaging and is thus considered more effective in identifying prolapsing mitral valve segments.[45] 3D-TEE, however, can simulate the surgeon’s view of the mitral valve and has become an essential tool in the intraoperative setting (see the image below).[46]



View Image

Mitral Valve Prolapse. Three-dimensional (3D) transesophageal echocardiogram (TEE) image (surgeon's view) of the mitral valve demonstrating prolapse o....

Pepi et al looked at data from 112 consecutive patients with severe mitral regurgitation secondary to MVP who underwent 2D and 3D TTE preoperatively with 2D and 3D TEE intraoperatively to localize the site of prolapse.[47]  When results from the imaging studies were compared to the "gold standard" of the intraoperative surgical findings, the investigators found the most accurate modality was 3D TEE (96% accurate); the least accurate was with 2D TTE (77% accurate).[47]

Other Imaging Studies

Cardiac magnetic resonance imaging (cMRI)

CMRI represents a relatively novel noninvasive imaging method that can identify mitral valve prolapse (MVP) with a sensitivity and specificity of 100%, using 2D transthoracic echocardiography (TTE) as the gold standard.[48] CMRI can also serve as an important tool for follow-up of patients with MVP-related moderate to severe mitral regurgitation (MR) for quantitative assessment of ventricular volumes and function.[49] It can also provide insight into the biology of the mitral valve; CMRI has shown gadolinium enhancement in both the mitral valve and papillary muscle tips in patients with MVP but not in healthy subjects without MVP.[48] It is postulated that the papillary muscle is altered in MVP by repetitive traction exerted by the prolapsing leaflets.[50] CMRI-determinants of MVP-related MR include anterior leaflet length, posterior leaflet displacement, and the presence of flail leaflets.[49]

In a study that used CMRI for identifying the structural hallmarks of patients with MVP who had complex ventricular arrhythmias, Basso et al found that all the patients had CMRI findings of left ventricular fibrosis at the level of the papillary muscles and in the inferobasal regions.[35] These findings correlated with autopsy findings of sudden cardiac death (SCD) in patients with MVP.

Contrast ventriculography

Contrast ventriculography can also help define MVP with or without MR. However, with the advent of echocardiography, this study is rarely necessary.

Chest radiography

Radiographs may demonstrate the progression from asymptomatic to chronic, severe MR, with the development of cardiomegaly secondary to left atrial and left ventricular dilatation and evidence of heart failure.

Histologic Findings

Gross pathologic specimens of myxomatous mitral valves reveal thickening of the leaflets and chords, interchordal hooding of the leaflets; sometimes, there is evidence of ruptured chorae.[51]

Histologically, valves that are affected by myxomatous changes show the middle layer of the valve leaflet (the spongiosa) having accumulated proteoglycans and glycosaminoglycans with reduction in collagen staining.[52] The tensile strength of the leaflet is affected by this accumulation of proteoglycans.

Medical Care

Medical therapy has a limited role in patients with primary mitral regurgitation (MR) from mitral valve prolapse (MVP) because the underlying process is a degenerative valve that requires intervention. Medical management should be focused on treating any concomitant hypertension and/or heart failure with guideline-directed medical therapy, especially in patients with reduced left ventricular (LV) ejection fraction (EF).

Early referral to cardiothoracic surgery in a center experienced with mitral valve interventions is recommended.

Asymptomatic patients with minimal disease

Asymptomatic patients with minimal mitral valve disease should be reassured of their benign prognosis. They should undergo initial echocardiography for risk stratification. If no clinically significant MR and thin leaflets are observed, clinical examinations and echocardiographic studies can be scheduled every 3-5 years. If echocardiography demonstrates leaflet thickening, which is considered a high-risk feature, these patients should be followed with annual echocardiograms.

Encourage these patients to pursue a normal, unrestricted lifestyle, including vigorous exercise.

Different measures may be aimed at orthostatic intolerance. A trial of beta-blockers for symptomatic relief can be recommended in patients with palpitations or other symptoms of an enhanced adrenergic drive. Abstinence from stimulants such as caffeine, alcohol, and cigarettes is also recommended. An ambulatory 24-hour Holter monitor may be useful for detecting supraventricular and/or ventricular arrhythmias.

Patients with evidence of progression to severe MR

If it is unclear whether or not the patient is asymptomatic with the degree of MR, a treadmill stress test for exercise tolerance can be performed. That is, have the patient demonstrate that they can walk vigorously without symptoms.

Asymptomatic patients with moderate-to-severe MR and LV enlargement, especially those with atrial fibrillation and/or pulmonary hypertension, should undergo surgery before LV function deteriorates. Echocardiographic criteria for severe MR include a vena contracta width more than 0.7 cm, a large central MR jet (area >40% of the left atrium), pulmonary vein reversal, an effective regurgitant orifice more than 40 mm2, a regurgitant fraction over 50%, and a regurgitant volume over 60 mL.[53]

According to the 2020 American College of Cardiology/American Heart Association (ACC/AHA), guidelines on the treatment of patients with valvular heart disease, mitral valve surgery is indicated for asymptomatic patients with chronic severe primary MR and an LVEF of 60% or less and/or a LV end-systolic dimension (LVESD) of 40 mm or more (class 1 recommendation).[54] These guidelines also recommend mitral valve intervention in symptomatic patients with severe primary MR irrespective of the LV systolic function (class 1 recommendation).[54]

In asymptomatic patients with severe primary MR, an LVEF of at least 60%, and an LVESD of 40 mm or less, it is reasonable to pursue mitral valve repair if the chance of successful and durable repair without residual MR is over 95% with an expected mortality below 1%, and the procedure can be completed at a primary or comprehensive valve center (class 2a recommendation).[54] In asymptomatic patients with severe primary MR and normal LV systolic function (LVEF >60%, LVESD < 40 mm) but who have progressive increase in the LV size or a decrease in LVEF on three or more serial imaging studies, then mitral valve surgery can be considered (class 2b recommendation).[54]

For patients who are surgical candidates and whose primary anatomic etiology of their MR is a degenerative disease, the ACC/AHA guidelines recommend mitral valve repair over mitral valve replacement if the repair will be successful and durable (class 1 recommendation).[54]

For patients with high or prohibitive surgical risk but otherwise meet criteria for mitral valve intervention, consideration can be given for a transcatheter-based approach with edge-to-edge repair if the valve anatomy is favorable and the patient's life expectancy is at least 1 year (class 2a recommendation).[54]

Patients with MVP and neurologic findings

Antithrombotic therapy is not recommended in patients without a history of systemic embolism, ischemic stroke, or atrial fibrillation. However, a thorough evaluation for atrial fibrillation should be undertaken with consideration for longer-term (eg, 30 day) monitoring.[55, 56] For secondary prevention, in the absence of demonstrable atrial fibrillation, antiplatelet therapy with aspirin at a dosage of 75-325 mg/day or clopidogrel 75 mg/day may be considered.

Recommend cessation of smoking and oral contraceptive use to prevent a hypercoagulable state.

Embolic events in patients with MVP and mild MR presumed to be secondary to paroxysmal atrial fibrillation should be treated according to general guidelines for atrial fibrillation.

Infective endocarditis (IE) prophylaxis

Endocarditis prophylaxis is no longer recommended by the ACC/AHA guidelines for all patients with MVP, except those with a prior episode of endocarditis or if surgical or transcatheter repair or replacement has occurred.[53, 57, 58]

Follow-up

Depending on the MVP severity, affected patients may be monitored regularly with a careful history, physical examination, and echocardiography when indicated.

If symptoms or physical findings change during outpatient care, echocardiography or other studies (ie, Holter monitor, radiography) may be indicated.

Surgical Care

The timing of surgery for mitral valve prolapse (MVP) should consider the anatomy of the valve lesion, the likelihood of a successful repair, and the local surgical experience.

In a study of 443 patients who underwent mitral valve repair for MVP, investigators found that preoperative atrial fibrillation was a predictor of long-term mortality and postoperative left ventricular dysfunction.[59] The investigators suggested that mitral valve repair surgery before the onset of atrial fibrillation may improve postoperative outcomes.

In a retrospective study of 1218 patients who underwent mitral valve repair for isolated degenerative mitral regurgitation (MR), postoperative MR recurrence was associated with adverse left ventricular remodeling and late death.[60] The investigators found a marked decrease in the incidence of MR recurrence following the first year after the intervention. They recommended that patients with complex MVP be informed of the risk of recurrent MR during the first year after degenerative mitral valve repair.[60]

See the surgical management discussion in the Medscape Drugs & Diseases article Mitral Regurgitation (Mitral Insufficiency).

Diet and Activity

Diet

There is no special diet that prevents or treats mitral valve prolapse (MVP); however, in patients who develop congestive heart failure symptoms, a low-salt diet is recommended to prevent acute congestive heart failure and hospitalizations.

Activity

Patients with MVP are encouraged to exercise and maintain physical activity. However, patients with the following conditions are instructed to avoid competitive exercise[61] :

Theoretically, heavy weightlifting may cause increased left ventricular wall tension and chordal extension, worsening prolapse, and regurgitation. Therefore, patients with MVP should avoid heavy weightlifting.

2017 ASE/ACMR Guidelines for Noninvasive Evaluation of Native Valve Regurgitation

In 2017, the American Society of Echocardiography (ASE) and the Society for Cardiovascular Magnetic Resonance (SCMR) jointly released their recommendations for the noninvasive evaluation of native valvular regurgitation.[62]

General considerations regarding echocardiography in the evaluation of valvular regurgitation

Echocardiographic parameters for the thorough assessment of valvular regurgitation include the following[62] :

Important considerations in the evaluation of MR

Once MR is identified, initiate an assessment of the mitral valve anatomy to establish the regurgitation mechanism (specify and report), classify whether the MR is primary or secondary (functional), particularly in MR that is more than mild.[62]

The ASE/SCMR note there does not exist a single Doppler and echocardiographic parameter precise enough to quantify MR in individual patients; instead, the combination of multiple parameters are needed to accurately assess MR severity. In the setting of multiple concordant parameters, there is a high probability of ascertaining MR severity, particularly for mild or severe MR.[62]

It is important to evaluate LV/LA volumes, as indexed to BSA, for accurate identification of enlarged chambers. It is unusual to have normal chamber volumes in chronic severe MR.[62]

When using color Doppler to assess MR severity, always evaluate the three jet components of flow convergence, size, and jet area, as well as the MR jet direction. Note very eccentric jets that hug the atrial wall—the jet area will underestimate the MR severity and be unusable.[62]

It is essential to evaluate the MR duration. Keep in mind that when MR is limited to late systole (mitral valve prolapse [MVP]) or early systole (ventricular dyssynchrony), it is generally not severe. However, there is the potential for its misinterpretation as severe when it is based only on single-color frame measurements (eg, proximal jet width [VC], proximal isovelocity surface area [PISA]).[62]

In the setting of unclear MR severity when using qualitative and semiquantitatiive parameters, obtain quantitative measurements of MR severity, as long as the data quality is good.[62]

Note the systemic BP and MR jet velocity; color flow jets and jet momentum (Av2) are proportional.[62] In the setting of hypertension, severe aortic stenosis, or significant LV outflow tract (OT) obstruction, a high velocity (eg, >6 m/sec) MR jet often appears large on color Doppler even though the effective regurgitant orifice area (EROA) or regurgitant volume [RVol] is small.

It is more clinically challenging to diagnosis acute severe MR caused by flail MV or ruptured papillary muscle than diagnosing chronic severe MR, particularly when using color Doppler (very eccentric MR and low MR velocity, short duration, tachycardia).[62] Maintain a high level of clinical suspicion if conventional echocardiography/Doppler parameters indicate significant MR, and use a low threshold for TEE performance.

The ASE/SCMR recommend additional testing with TEE or cardiac magnetic resonance imaging (CMRI) in the following settings[62] :

2020 ACC/AHA Valvular Heart Disease Guidelines

The American College of Cardiology (ACC) and American Heart Association (AHA) released their updated recommendations on managing valvular heart disease in December 2020.[54, 63] Ten key messages are outlined below.

Valvular heart disease (VHD) stages (stages A-D) in patients should be classified based on symptoms, valve anatomy, severity of valve dysfunction, and response of the ventricle and pulmonary circulation.

When evaluating patients with VHD, findings from the history and physical examination (PE) should be correlated with those from noninvasive testing (ie, electrocardiography [ECG], chest x-ray, transthoracic echocardiography [TTE]). If conflict exists between results on the PE and that of initial noninvasive studies, consider obtaining further noninvasive (computed tomography [CT], cardiac magnetic resonance imaging [CMRI], stress testing) or invasive (transesophageal echocardiography [TEE], cardiac catheterization) studies to decide the optimal treatment strategy.

In the setting of VHD and atrial fibrillation (AF) (except for patients with rheumatic mitral stenosis [MS] or a mechanical prosthesis), the decision to use oral anticoagulation with either a vitamin K antagonist (VKA) or a non-VKA anticoagulant to prevent thromboembolic events should be a shared decision-making process based on the CHA2DS2-VASc score (congestive heart failure [CHF], hypertension, age ≥75 years, diabetes mellitus, previous stroke/transient ischemic attack/thromboembolic event, vascular disease, age 65-74 years, sex). Oral anticoagulation with a VKA should be given to those with rheumatic MS or a mechanical prosthesis and AF.

All those with severe VHD under consideration for valve intervention should be evaluated by a multidisciplinary team, either with a referral or in consultation with a primary or comprehensive valve center.

Treatment of severe aortic stenosis (AS) with either a transcatheter or surgical valve prosthesis should be based primarily on symptoms or reduced ventricular systolic function. Consider earlier intervention if indicated by the results of exercise testing, biomarkers, rapid progression, or the presence of very severe stenosis.

Expanded indications for transcatheter aortic valve implantation (TAVI) are a result of findings from multiple randomized trials of TAVI versus surgical aortic valve replacement (SAVR). The selection of intervention type for patients with severe AS should be a shared decision-making process that considers the lifetime risks and benefits associated with the valve type (mechanical vs bioprosthetic) and approach type (transcatheter vs surgical).

Indications for intervention for valvular regurgitation are symptomatic relief and prevention of the irreversible long-term consequences of left ventricular volume overload. Lowered thresholds for intervention than they were previously are owing to more durable treatment options and lower procedural risks.

A mitral transcatheter edge-to-edge repair benefits patients with severely symptomatic primary mitral regurgitation (MR) who are at high or prohibitive surgical risk, as well as benefits a select subset of patients with secondary MR who remain severely symptomatic despite guideline-directed management and heart failure therapy.

Patients who present with severe symptomatic isolated tricuspid regurgitation, commonly associated with device leads and AF, may benefit from surgical intervention to reduce symptoms and recurrent hospitalizations if performed before the onset of severe right ventricular dysfunction or hepatic and renal end-organ damage.

Bioprosthetic valve dysfunction may occur because of either degeneration of the valve leaflets or valve thrombosis. Catheter-based treatment for prosthetic valve dysfunction is reasonable in selected patients for bioprosthetic leaflet degeneration or paravalvular leak in the absence of active infection.

For more information, please go to Aortic Stenosis, Aortic Regurgitation, Mitral Stenosis, Mitral Regurgitation, and Tricuspid Regurgitation.

Author

Priyanka Ghosh, DO, Fellow in Cardiovascular Disease, Guthrie Robert Packer Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Saurabh Sharma, MD, FACC, FACP, FASE, RPVI, Associate Non-Invasive Cardiologist, Director, Cardiovascular Prevention and Lipid Clinic, Program Director, Internal Medicine Residency Program, Guthrie Medical Group, Guthrie Robert Packer Hospital; Clinical Assistant Professor of Cardiology, Geisinger Commonwealth School of Medicine

Disclosure: Nothing to disclose.

Specialty Editors

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Terrence X O'Brien, MD, MS, FACC, Professor of Medicine/Cardiology, Director, Clinical Cardiovascular Research, Medical University of South Carolina College of Medicine; Director, Echocardiography Laboratory, Veterans Affairs Medical Center of Charleston

Disclosure: Nothing to disclose.

Additional Contributors

Justin D Pearlman, MD, ME, PhD, FACC, MA, Chief, Division of Cardiology, Director of Cardiology Consultative Service, Director of Cardiology Clinic Service, Director of Cardiology Non-Invasive Laboratory, Chair of Institutional Review Board, University of California, Los Angeles, David Geffen School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

Alan D Forker, MD Professor of Medicine, University of Missouri at Kansas City School of Medicine; Director, Outpatient Lipid Diabetes Research, MidAmerica Heart Institute of St Luke's Hospital

Disclosure: Nothing to disclose.

References

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Mitral Valve Prolapse. Three-dimensional (3D) transesophageal echocardiogram (TEE) image (surgeon's view) of the mitral valve demonstrating prolapse of the P1 and P2 scallop (annotated in red). Ruptured chords are also visualized.

Mitral Valve Prolapse. Three-dimensional (3D) transesophageal echocardiogram (TEE) image (surgeon's view) of the mitral valve demonstrating prolapse of the P1 and P2 scallop (annotated in red). Ruptured chords are also visualized.