(See also Overview of Cardiac Valvular Disorders Overview of Cardiac Valvular Disorders Any heart valve can become stenotic or insufficient (also termed regurgitant or incompetent), causing hemodynamic changes long before symptoms. Most often, valvular stenosis or insufficiency... read more .)
In mitral stenosis, mitral valve leaflets become thickened and immobile and the mitral orifice becomes narrowed due to fusion of the commissures and the presence of shortened, thickened and matted chordae.
The most common cause is rheumatic fever Rheumatic Fever Rheumatic fever is a nonsuppurative, acute inflammatory complication of group A streptococcal pharyngeal infection, causing combinations of arthritis, carditis, subcutaneous nodules, erythema... read more , even though many patients do not recall the disorder. Women comprise 80% of cases. In regions with high prevalence of rheumatic fever, patients often present by age 30, usually with commissural fusion of pliable, noncalcified leaflets. In regions with low prevalence, patients typically present between ages 50 and 70 and with calcified fibrotic leaflets with subvalvular involvement in addition to commissural fusion.
A rare but increasing cause occurs in older patients with mitral annular calcification with extension of calcification into the leaflets, causing them to stiffen and not open fully. Occasionally, mitral stenosis is congenital or occurs after radiation therapy. If the valve cannot close completely, mitral regurgitation Mitral Regurgitation Mitral regurgitation (MR) is incompetency of the mitral valve causing flow from the left ventricle (LV) into the left atrium during ventricular systole. MR can be primary (common causes are... read more (MR) may coexist with mitral stenosis. Patients with mitral stenosis due to rheumatic fever may also have lesions of the aortic or tricuspid valve or both.
Left atrial (LA) size and pressure increase progressively to compensate for mitral stenosis; pulmonary venous and capillary pressures also increase and may cause secondary pulmonary hypertension Symptoms and Signs Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels become constricted... read more , leading to right ventricular (RV) heart failure RV failure Heart failure (HF) is a syndrome of ventricular dysfunction. Left ventricular failure causes shortness of breath and fatigue, and right ventricular failure causes peripheral and abdominal fluid... read more , tricuspid regurgitation Tricuspid Regurgitation Tricuspid regurgitation (TR) is insufficiency of the tricuspid valve causing blood flow from the right ventricle to the right atrium during systole. The most common cause is dilation of the... read more , and pulmonic regurgitation Pulmonic Regurgitation Pulmonic (pulmonary) regurgitation (PR) is incompetency of the pulmonic valve causing blood flow from the pulmonary artery into the right ventricle during diastole. The most common cause is... read more . Rate of progression varies.
LA enlargement predisposes to atrial fibrillation Atrial Fibrillation Atrial fibrillation is a rapid, irregularly irregular atrial rhythm. Symptoms include palpitations and sometimes weakness, effort intolerance, dyspnea, and presyncope. Atrial thrombi may form... read more (AF), a risk factor for thromboembolism. The faster heart rate and loss of atrial contraction with onset of AF often lead to sudden worsening of symptoms.
Symptoms and Signs of Mitral Stenosis
Symptoms of mitral stenosis correlate poorly with disease severity because the disease often progresses slowly, and patients unconsciously reduce their activity. Many patients are asymptomatic until they become pregnant or AF develops. Initial symptoms are usually those of heart failure (eg, exertional dyspnea, orthopnea, paroxysmal nocturnal dyspnea, fatigue).
Symptoms typically do not appear until 15 to 40 years after an episode of rheumatic fever. In medically underserved countries, young children may become symptomatic because streptococcal infections may not be treated with antibiotics and recurrent infections are common.
Paroxysmal or chronic atrial fibrillation further reduces blood flow into the left ventricle (LV), precipitating pulmonary edema Pulmonary Edema Pulmonary edema is acute, severe left ventricular failure with pulmonary venous hypertension and alveolar flooding. Findings are severe dyspnea, diaphoresis, wheezing, and sometimes blood-tinged... read more and acute dyspnea when ventricular rate is poorly controlled. AF may also cause palpitations. In up to 15% of patients not taking anticoagulants, it causes systemic embolism with symptoms of stroke or other organ ischemia.
Less common symptoms include hemoptysis due to rupture of small pulmonary vessels and pulmonary edema, particularly during pregnancy when blood volume increases. Hoarseness due to compression of the left recurrent laryngeal nerve by a dilated LA or pulmonary artery (Ortner syndrome) and symptoms of pulmonary hypertension Symptoms and Signs Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels become constricted... read more and RV failure RV failure Heart failure (HF) is a syndrome of ventricular dysfunction. Left ventricular failure causes shortness of breath and fatigue, and right ventricular failure causes peripheral and abdominal fluid... read more may also occur.
Mitral stenosis may cause signs of cor pulmonale Cor Pulmonale Cor pulmonale is right ventricular enlargement secondary to a lung disorder that causes pulmonary artery hypertension. Right ventricular failure follows. Findings include peripheral edema, neck... read more . The classic facial appearance in mitral stenosis, a plum-colored malar flush, occurs only when cardiac output is low and pulmonary hypertension is severe; cause is cutaneous vasodilation and chronic hypoxemia.
Occasionally, the initial symptoms and signs of mitral stenosis are those of an embolic event such as stroke. Endocarditis is rare in mitral stenosis unless mitral regurgitation is also present.
Palpation may detect palpable 1st and 2nd heart sounds (S1 and S2). S1 is best palpated at the apex, and S2 at the upper left sternal border. The pulmonic component of S2 (P2) is responsible for the impulse and results from pulmonary hypertension. An RV impulse (heave) palpable at the left sternal border may accompany jugular venous distention when pulmonary hypertension is present and RV diastolic dysfunction develops.
Early diastolic opening snap
Low-pitched decrescendo-crescendo rumbling diastolic murmur
Auscultatory findings in mitral stenosis include a loud S1 caused by the leaflets of a stenotic mitral valve closing abruptly (M1); it is heard best at the apex. S1 may be absent when the valve is heavily calcified and immobile. A normally split S2 with an exaggerated P2 due to pulmonary hypertension is also heard (see table Distinguishing the Murmurs of Tricuspid Stenosis and Mitral Stenosis Distinguishing the Murmurs of Tricuspid Stenosis and Mitral Stenosis Mitral stenosis is narrowing of the mitral orifice that impedes blood flow from the left atrium to the left ventricle. The (almost) invariable cause is rheumatic fever. Common complications... read more ).
Most prominent is an early diastolic opening snap as the leaflets billow into the LV, which is loudest close to left lower sternal border; it is followed by a low-pitched decrescendo-crescendo rumbling diastolic murmur, heard best with the bell of the stethoscope at the apex (or over the palpable apex beat) at end-expiration when the patient is in the left lateral decubitus position. The opening snap may be soft or absent if the mitral valve is calcified; the snap moves closer to S2 (increasing duration of the murmur) as mitral stenosis becomes more severe and LA pressure increases.
The diastolic murmur increases after a Valsalva maneuver (when blood pours into the LA), after exercise, and in response to maneuvers that increase afterload (eg, squatting, isometric handgrip). The murmur may be softer or absent when an enlarged RV displaces the LV posteriorly and when other disorders (pulmonary hypertension, right-sided valve abnormalities, AF with fast ventricular rate) decrease blood flow across the mitral valve. The presystolic crescendo is caused by increased flow with atrial contraction. However, the closing mitral valve leaflets during LV contraction may also contribute to this finding but only at the end of short diastoles when LA pressure is still high.
Diastolic murmurs that may coexist with the MS murmur are:
Early diastolic murmur of coexisting aortic regurgitation Aortic Regurgitation Aortic regurgitation (AR) is incompetency of the aortic valve causing backflow from the aorta into the left ventricle during diastole. Causes include valvular degeneration and aortic root dilation... read more (AR), which may be conducted to the apex
Diastolic flow murmur in the presence of severe MR
Mid-diastolic murmur of obstructing left atrial myxoma or ball thrombus (rare)
Graham Steell murmur (a soft decrescendo diastolic murmur heard best along the left sternal border and caused by pulmonic regurgitation Pulmonic Regurgitation Pulmonic (pulmonary) regurgitation (PR) is incompetency of the pulmonic valve causing blood flow from the pulmonary artery into the right ventricle during diastole. The most common cause is... read more secondary to severe pulmonary hypertension)
Diagnosis of Mitral Stenosis
Diagnosis of mitral stenosis is suspected clinically and confirmed by echocardiography Echocardiography This photo shows a patient having echocardiography. This image shows all 4 cardiac chambers and the tricupsid and mitral valves. Echocardiography uses ultrasound waves to produce an image of... read more . Typically, 2-dimensional echocardiography shows abnormal valve and subvalvular structures. It also provides information about the degree of valvular calcification and stenosis and LA size. Doppler echocardiography provides information about the transvalvular gradient and pulmonary artery pressure. The normal area of the mitral valve orifice is 4 to 5 cm2.
Severity of mitral stenosis is characterized echocardiographically as
Moderate: Valve area > 1.5 to 2.5 cm2 or diastolic pressure half-time < 150 msec
Severe: Valve area ≤ 1.5 cm2 or diastolic pressure half-time ≥ 150 msec; symptoms are often present
However, the relationship between the area of the valve orifice and symptoms is not always consistent. Color Doppler echocardiography detects associated MR. Transesophageal echocardiography can be used to detect or exclude small LA thrombi, especially those in the LA appendage, which usually cannot be seen transthoracically. Transesophageal echocardiography also can better assess mitral regurgitation when mitral calcification causes acoustic shadowing of the left atrium.
An ECG and chest x-ray are usually obtained.
The ECG Electrocardiography The standard electrocardiogram (ECG) provides 12 different vector views of the heart’s electrical activity as reflected by electrical potential differences between positive and negative electrodes... read more may show LA enlargement, manifest as a P wave lasting > 0.12 msec with prominent negative deflection of its terminal component (duration: > 0.04 msec; amplitude: > 0.10 mV) in V1; broad, notched P waves in lead II; or both. Right axis QRS deviation and tall R waves in V1 suggest RV hypertrophy.
Chest x-ray Chest x-ray Chest imaging includes use of plain x-rays, computed tomography (CT) scanning, magnetic resonance imaging (MRI), nuclear scanning, including positron emission tomography (PET) scanning, and... read more usually shows straightening of the left cardiac border due to a dilated LA appendage, and widening of the carina. With barium in the esophagus, the lateral chest x-ray will show the dilated LA displacing the esophagus posteriorly. The main pulmonary artery (trunk) may be prominent; the descending right pulmonary artery diameter is ≥ 16 mm if pulmonary hypertension is significant. The upper lobe pulmonary veins may be dilated. A double shadow of an enlarged LA may be seen along the right cardiac border. Horizontal lines in the lower posterior lung fields (Kerley B lines) indicate interstitial edema associated with high LA pressure.
Exercise testing Stress Testing In stress testing, the heart is monitored by electrocardiography (ECG) and often imaging studies during an induced episode of increased cardiac demand so that ischemic areas potentially at risk... read more helps quantify symptoms. Further information can be obtained from stress echocardiography evaluation of changes in valve gradient and pulmonary pressure.
Cardiac catheterization Cardiac Catheterization Cardiac catheterization is the passage of a catheter through peripheral arteries or veins into cardiac chambers, the pulmonary artery, and coronary arteries and veins. Cardiac catheterization... read more , indicated only for perioperative assessment of coronary artery disease (CAD) before surgical repair, can confirm elevated LA and pulmonary artery pressures, mitral gradient and valve area.
Prognosis for Mitral Stenosis
The natural history of mitral stenosis varies, but the interval between onset of symptoms and severe disability is about 7 to 9 years. Outcome is affected by the patient’s preprocedural age and functional status, pulmonary hypertension, and degree of mitral regurgitation Mitral Regurgitation Mitral regurgitation (MR) is incompetency of the mitral valve causing flow from the left ventricle (LV) into the left atrium during ventricular systole. MR can be primary (common causes are... read more . Symptomatic results of balloon or surgical commissurotomy are equivalent in patients with valves that are not calcified. However, after a variable period of time, function deteriorates in most patients due to restenosis, and valve replacement may become necessary. Risk factors for death are atrial fibrillation Atrial Fibrillation Atrial fibrillation is a rapid, irregularly irregular atrial rhythm. Symptoms include palpitations and sometimes weakness, effort intolerance, dyspnea, and presyncope. Atrial thrombi may form... read more and pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels become constricted... read more . Cause of death is most commonly heart failure or pulmonary or cerebrovascular embolism.
Treatment of Mitral Stenosis
Diuretics and sometimes beta-blockers or calcium channel blockers
Anticoagulation for AF
Commissurotomy or valve replacement
Asymptomatic patients with mitral stenosis require no treatment other than appropriate prophylaxis against rheumatic fever Antibiotic prophylaxis Rheumatic fever is a nonsuppurative, acute inflammatory complication of group A streptococcal pharyngeal infection, causing combinations of arthritis, carditis, subcutaneous nodules, erythema... read more recurrence. Surveillance with serial TTE is important, because RV enlargement and rise in RV systolic pressure can occur without patients noticing a change in functional state, and without a decrease in mitral valve area. Early intervention may relieve pulmonary hypertension before it becomes permanent.
Mildly symptomatic patients usually respond to diuretics and, if sinus tachycardia or AF is present, to beta-blockers or calcium channel blockers, which can control ventricular rate.
Anticoagulation with a vitamin K antagonist (not a direct-acting oral anticoagulant [DOAC]) is indicated to prevent thromboembolism if patients have or have had AF, embolism, or a left atrial clot. Extended restoration of sinus rhythm is rarely possible. Anticoagulation may be considered in the presence of dense spontaneous contrast or an enlarged left atrium (M-mode diameter > 50 mm), but there are no good data to support this practice. All patients should be encouraged to continue at least low levels of physical exercise despite exertional dyspnea.
Antibiotic prophylaxis against endocarditis is no longer recommended except for patients who have had valve replacement (see table Recommended Endocarditis Prophylaxis During Oral-Dental or Respiratory Tract Procedures Recommended Endocarditis Prophylaxis During Oral-Dental or Respiratory Tract Procedures* Infective endocarditis is infection of the endocardium, usually with bacteria (commonly, streptococci or staphylococci) or fungi. It may cause fever, heart murmurs, petechiae, anemia, embolic... read more ).
Timing of intervention
Moderate mitral stenosis intervention may be indicated when cardiac surgery is required for other indications. Patients who are symptomatic and have exercise-induced mean transmitral gradient > 15 mm Hg or pulmonary capillary occlusion pressure > 25 mm Hg may be considered for percutaneous balloon commissurotomy.
Severe mitral stenosis intervention is indicated when any symptoms are present if the valve is suitable for percutaneous balloon commissurotomy (may be considered in asymptomatic patients if pulmonary pressure is > 50 mm Hg or there is new onset AF). Cardiac surgery is only pursued when symptoms are severe and for patients who are not candidates for percutaneous balloon commissurotomy or require other cardiac operations or do not have access to the percutaneous procedure.
Choice of intervention
Percutaneous balloon commissurotomy is the procedure of choice for younger patients and for patients without heavily calcified valve commissures, subvalvular distortion, LA thrombi, or moderate or severe MR (see Table Grading of Mitral Regurgitation Grading of Mitral Regurgitation Mitral regurgitation (MR) is incompetency of the mitral valve causing flow from the left ventricle (LV) into the left atrium during ventricular systole. MR can be primary (common causes are... read more —1 Treatment reference Mitral stenosis is narrowing of the mitral orifice that impedes blood flow from the left atrium to the left ventricle. The (almost) invariable cause is rheumatic fever. Common complications... read more ). In this fluoroscopic- and echocardiographic-guided procedure, a transvenous catheter with an inflatable distal balloon is passed transseptally from the right atrium to the LA and inflated to separate fused mitral valve commissures. Outcomes are equivalent to those of more invasive procedures. Complications are uncommon but include MR, embolism, and tamponade Cardiac Tamponade Cardiac tamponade is accumulation of blood in the pericardial sac of sufficient volume and pressure to impair cardiac filling. Patients typically have hypotension, muffled heart tones, and distended... read more .
Surgical commissurotomy may be used in patients with severe subvalvular disease, valvular calcification, or LA thrombi. In this procedure, fused mitral valve leaflets are separated using a dilator passed through the left ventricle (closed commissurotomy) via a thoracotomy, or by direct vision (open commissurotomy) via a sternotomy. Choice of procedure is based on surgeon’s experience and the morphology of the valve, although closed valvotomy is now done less frequently. Because of its greater risks, surgery is usually deferred until symptoms reach New York Heart Association class III (see table NYHA Classification of Heart Failure New York Heart Association (NYHA) Classification of Heart Failure Heart failure (HF) is a syndrome of ventricular dysfunction. Left ventricular failure causes shortness of breath and fatigue, and right ventricular failure causes peripheral and abdominal fluid... read more ). During surgery, some clinicians ligate the left atrial appendage to reduce thromboembolism.
Valve replacement is confined to patients with severe morphologic changes that make the valve unsuitable for balloon or surgical commissurotomy. Lifelong anticoagulation with warfarin is required in patients with a mechanical valve to prevent thromboembolism. A mitral bioprosthetic valve requires anticoagulation with warfarin for 3 to 6 months postoperatively (see also Anticoagulation for patients with a prosthetic cardiac valve Anticoagulation for patients with a prosthetic cardiac valve Any heart valve can become stenotic or insufficient (also termed regurgitant or incompetent), causing hemodynamic changes long before symptoms. Most often, valvular stenosis or insufficiency... read more ). Direct-acting oral anticoagulants (DOAC) are ineffective and should not be used.
When the etiology is annular calcification, there is no benefit from percutaneous balloon commissurotomy because there is no commissural fusion. Furthermore, surgical valve replacement is technically demanding because of the annular calcification and often high risk because many patients are older and have comorbidities. Therefore, intervention is delayed until symptoms become severe despite use of diuretic and rate control drugs. Preliminary experience in inoperable patients suggests benefit from implantation of a transcatheter aortic valve replacement (TAVR) bioprosthesis in the mitral position.
1. Otto CM, Nishimura RA, Bonow RO, et al: 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation 143(5):e35–e71, 2021. doi: 10.1161/CIR.0000000000000932
Mitral stenosis is almost always caused by rheumatic fever.
Pulmonary hypertension and atrial fibrillation may develop.
The risk of thromboembolism in patients with atrial fibrillation and mitral stenosis is very high and is treated with a vitamin K antagonist, not a direct-acting oral anticoagulant.
Heart sounds include a loud S1 and an early diastolic opening snap followed by a low-pitched decrescendo-crescendo rumbling diastolic murmur, heard best at the apex at end-expiration when the patient is in the left lateral decubitus position; the murmur increases after a Valsalva maneuver, exercise, squatting, and isometric handgrip.
Mildly symptomatic patients usually respond to diuretics and, if sinus tachycardia or atrial fibrillation is present, to beta-blockers or calcium channel blockers for rate control.
Severely symptomatic patients and those with evidence of pulmonary hypertension require commissurotomy or valve replacement.
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