Mitral Stenosis Treatment & Management

  • Author: Claudia Dima, MD; Chief Editor: Richard A Lange, MD   more...
 
Updated: Nov 1, 2010
 

Medical Care

The goal of medical treatment for mitral stenosis is to reduce recurrence of rheumatic fever, provide prophylaxis for infective endocarditis, reduce symptoms of pulmonary congestion (eg, orthopnea, paroxysmal nocturnal dyspnea), control the ventricular rate if atrial fibrillation is present, and prevent thromboembolic complications.

  • Because rheumatic fever is the primary cause of mitral stenosis, secondary prophylaxis against group A beta-hemolytic streptococci (GAS) is recommended.[3] Duration of prophylaxis depends on the number of previous attacks, the time elapsed since the last attack, the risk of exposure to GAS infections, the age of the patient, and the presence or absence of cardiac involvement. Penicillin is the agent of choice for secondary prophylaxis, but sulfadiazine or a macrolide or azalide are acceptable alternatives in individuals allergic to penicillin (Tables 1 and 2).
  • A recent study done in Europe on 315 patients with rheumatic mitral stenosis showed a significantly slower progression of rheumatic mitral stenosis in patients treated with statins compared with patients not taking statins. These findings could have an important impact in the early medical therapy of patients with rheumatic heart disease.[4]
  • The current American Heart Association (AHA) recommendations[5] no longer suggest infective endocarditis prophylaxis for patients with rheumatic heart disease. However, the maintenance of optimal oral health care remains an important component of an overall healthcare program. For the relatively few patients with rheumatic heart disease in whom infective endocarditis prophylaxis remains recommended, such as those with prosthetic valves or prosthetic material used in valve repair, the current AHA recommendations should be followed. These recommendations advise the use of an agent other than a penicillin to prevent infective endocarditis in those receiving penicillin prophylaxis for rheumatic fever because oral alpha-hemolytic streptococci are likely to have developed resistance to penicillin.
  • Initial symptoms of pulmonary congestion can be safely treated by diuretics. Dietary sodium restriction and nitrates decrease preload and can be of additional benefit. Careful use of beta-blockers in patients with a normal sinus rhythm can prolong the diastolic filling time and thus decrease in left atrial pressure. In general, afterload reduction should be avoided as it can cause hypotension.
  • Atrial fibrillation is common in mitral stenosis and often leads to a rapid ventricular rate with reduced diastolic filling time and increased left atrial pressure. The ventricular rate can be slowed acutely by the administration of intravenous beta-blocker or calcium channel blocker therapy (diltiazem or verapamil). The rate and/or rhythm can be controlled long-term with oral beta-blockers, calcium channel blockers, amiodarone, or digoxin.
  • In the patient with mild mitral stenosis and recent-onset (< 6 mo) atrial fibrillation, conversion to sinus rhythm can be accomplished with pharmacologic agents or electrical cardioversion. In this circumstance, anticoagulation therapy should be given for at least 3 weeks prior to cardioversion. Alternatively, a TEE can be performed to exclude the presence of left atrial thrombus, prior to cardioversion. Patients who are successfully converted to sinus rhythm should receive long-term anticoagulation and antiarrhythmic drugs.
  • Surgical correction of the mitral stenosis is indicated if embolization is recurrent, despite adequate anticoagulation therapy.

Table 1. Duration of Secondary Rheumatic Fever Prophylaxis (Open Table in a new window)

CategoryDuration After Last AttackRating*
Rheumatic fever with carditis and residual heart disease (persistent valvular disease† )10 y or until age 40 y (whichever is longer); sometimes lifelong prophylaxisIC
Rheumatic fever with carditis but no residual heart disease (no valvular disease† )10 y or until age 21 y (whichever is longer)IC
Rheumatic fever without carditis5 y or until age 21 y (whichever is longer)IC
*Rating indicates classification of recommendation and level of evidence (eg, IC indicates Class I, level of Evidence C).



†Clinical or echocardiographic evidence.



Table 2. Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) (Open Table in a new window)

AgentDoseModeRating*
Benzathine penicillin GChildren 27 kg (60 lb): 600,000 U



Patients >27 kg: 1,200,000 every 4 wk†



IntramuscularIA
Penicillin V250 mg bidOralIB
SulfadiazineChildren 27 kg: 0.5 g qd



Patients >27 kg: 1 g qd



OralIB
Macrolide or azalide (for individuals allergic to penicillin and sulfadiazine)VariableOralIC
*Rating indicates classification of recommendation and level of evidence (eg, IA indicates Class I, level of Evidence A).



†In high-risk situations, administration every 3 weeks is justified and recommended.



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Surgical Care

Surgical therapy for mitral stenosis consists of mitral valvotomy (which can be either surgical or percutaneous) or mitral valve replacement. The surgical mitral valvotomy approach can be through an open or closed technique; the latter technique is rarely used, except in developing countries, and has largely been replaced by the percutaneous balloon valvotomy.[2]

Asymptomatic patients with moderate or severe mitral stenosis (mitral valve area < 1.5 cm2) and a suitable valve should be considered for percutaneous balloon valvuloplasty if the pulmonary arterial systolic pressure is ≥ 50 mm Hg at rest or ≥ 60 mm Hg with exercise, or pulmonary capillary wedge pressure is ≥ 25 mm Hg with exercise.[6]

Symptomatic patients with moderate or severe mitral stenosis (mitral valve area < 1.5 cm2) and suitable valve are also candidates for percutaneous balloon valvuloplasty.

If percutaneous balloon valvuloplasty is not an option, patients should be referred for surgical repair or mitral valve replacement.

  • Percutaneous balloon valvuloplasty
    • Percutaneous balloon valvuloplasty is the procedure of choice for patients with uncomplicated mitral stenosis. Patients with pliable, mobile, relatively thin, minimally calcified mitral leaflets with minimal or no subvalvular stenosis are good candidates for this procedure. A TEE should be performed prior to valvotomy to clearly define the valve anatomy and exclude the presence of a left atrial thrombus.
    • The echocardiographic scoring system (Wilkins score) has been used as a valuable tool for patient selection. Leaflet mobility, valvular thickening, valvular calcification, and subvalvular disease are each given a score of 0-4, with higher scores indicating more severe involvement. A total score of less than 8 results in good short- and long-term outcome with balloon valvuloplasty.
    • With percutaneous balloon valvuloplasty, a catheter is directed into the left atrium after transseptal puncture, and a balloon is directed across the valve and inflated in the orifice. This results in separation of the mitral leaflets. The valve size can be increased up to 2-2.5 cm2.
    • Improvement in symptoms is noted immediately following the procedure. If symptoms do not improve, the valvuloplasty was either ineffective or resulted in mitral regurgitation.
    • The short- and long-term prognoses are favorable compared with surgical valvotomy.
    • Balloon valvuloplasty offers certain advantages over surgical valvotomy, including avoidance of a thoracotomy and general anesthesia and their attendant complications.
    • The major contraindications to balloon valvuloplasty are the presence of thrombus in the left atrium or its appendage, moderate-to-severe mitral regurgitation, and an unfavorable valve morphology (ie, high Wilkins echo score).
    • Complications of a balloon mitral valvuloplasty include embolization, mitral regurgitation, ventricular rupture, residual atrial septal defect, stroke, and death.
  • Surgical valvotomy/valve replacement[7]
    • Open surgical commissurotomy allows direct visualization of the mitral valve.
    • Using current techniques, even severe regurgitant or stenotic valves can often be repaired, with good long-term results. Valves that are not suitable for repair can be replaced using either bioprosthetic or metallic prosthetic valves.
    • With bioprosthetic valves, the patient does not require anticoagulation, as long as he or she remains in sinus rhythm; however, 20-40% of these valves fail within 10 years, secondary to structural deterioration.
    • Mechanical valves are placed in young patients who do not have any contraindications for anticoagulation, and these valves are associated with good long-term durability.
    • Patients who have chronic atrial fibrillation and who undergo mitral valve surgery can have simultaneous Cox Maze procedure or pulmonary vein ablation, which helps to maintain sinus rhythm in up to 80% of the cases during the postoperative period.
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Consultations

A cardiology and/or cardiothoracic surgery consult may be necessary.

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Diet

The patient should start a low-salt diet if pulmonary vascular congestion is present.

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Activity

In most patients with mitral stenosis, recommendations for exercise are symptom limited. Patients should be encouraged to pursue a low-level aerobic exercise program for maintenance of cardiovascular fitness.

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Contributor Information and Disclosures
Author

Claudia Dima, MD  Cardiovascular Fellow, Department of Cardiology, Banner Good Samaritan Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Kenneth B Desser, MD  Clinical Professor, Director of Cardiology Fellowship, Banner Good Samaritan Medical Center, Phoenix, Arizona

Disclosure: Nothing to disclose.

Specialty Editor Board

L Michael Prisant, MD, FACC, FAHA  Cardiologist, Emeritus Professor of Medicine, Medical College of Georgia

L Michael Prisant, MD, FACC, FAHA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Clinical Pharmacology, American College of Forensic Examiners, American College of Physicians, American Heart Association, and American Medical Association

Disclosure: Boehringer-Ingelheim Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Steven J Compton, MD, FACC, FACP  Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals

Steven J Compton, MD, FACC, FACP is a member of the following medical societies: Alaska State Medical Association, American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, and Heart Rhythm Society

Disclosure: Nothing to disclose.

Amer Suleman, MD  Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD  Professor and Executive Vice Chairman, Department of Medicine, Director, Office of Educational Programs, University of Texas Health Science Center at San Antonio

Richard A Lange, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, and Association of Subspecialty Professors

Disclosure: Nothing to disclose.

References
  1. Marcus RH, Sareli P, Pocock WA, et al. The spectrum of severe rheumatic mitral valve disease in a developing country. Correlations among clinical presentation, surgical pathologic findings, and hemodynamic sequelae. Ann Intern Med. Feb 1 1994;120(3):177-83. [Medline].

  2. Bruce CJ, Nishimura RA. Newer advances in the diagnosis and treatment of mitral stenosis. Curr Probl Cardiol. Mar 1998;23(3):125-92. [Medline].

  3. [Guideline] Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation. Mar 24 2009;119(11):1541-51. [Medline].

  4. Antonini-Canterin F, Moura LM, Enache R, Leiballi E, Pavan D, Piazza R. Effect of hydroxymethylglutaryl coenzyme-a reductase inhibitors on the long-term progression of rheumatic mitral valve disease. Circulation. May 18 2010;121(19):2130-6. [Medline].

  5. [Guideline] Nishimura RA, Carabello BA, Faxon DP, Freed MD, Lytle BW, O'Gara PT. ACC/AHA 2008 Guideline update on valvular heart disease: focused update on infective endocarditis: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. Aug 19 2008;52(8):676-85. [Medline].

  6. Feldman T. Rheumatic Mitral Stenosis. Curr Treat Options Cardiovasc Med. Apr 2000;2(2):93-104. [Medline].

  7. Rahimtoola SH. Choice of Prosthetic Heart Valve in Adults An Update. J Am Coll Cardiol. Jun 1 2010;55(22):2413-2426. [Medline].

  8. Horstkotte D, Niehues R, Strauer BE. Pathomorphological aspects, aetiology and natural history of acquired mitral valve stenosis. Eur Heart J. Jul 1991;12 Suppl B:55-60. [Medline].

  9. [Guideline] Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr, Faxon DP, Freed MD, et al. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to revise the 1998 guidelines for the management of patients with valvular heart disease). Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. Sep 23 2008;52(13):e1-142. [Medline].

  10. Bonow RO, Otto CM. Valvular heart disease. In: Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 2. 8th ed. Philadelphia, PA: WB Saunders; 2008:1646-1657.

  11. Carabello BA. Modern management of mitral stenosis. Circulation. Jul 19 2005;112(3):432-7. [Medline].

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M-mode across the mitral valve showing a flat E-F slope resulting from elevated left atrial pressure throughout diastole due to a significant gradient across the mitral valve. Increased thickness and calcification of anterior leaflet of the mitral valve and decreased opening of the anterior and posterior leaflets in diastole are also shown.
Parasternal long-axis view demonstrating calcification and doming in diastole of the anterior valve leaflet and mild restriction in the opening of posterior mitral valve leaflet.
Apical 4-chamber view demonstrating restricted opening of the anterior and posterior mitral valve leaflet with diastolic doming of anterior leaflet with left atrial enlargement.
Transesophageal echocardiogram with continuous wave Doppler interrogation across the mitral valve demonstrating an increased mean gradient of 16 mm Hg consistent with severe mitral stenosis.
Apical 4-chamber view with color Doppler demonstrating aliasing in the atrial side of the mitral valve consistent with increased gradient across the valve. This figure also shows mitral regurgitation and left atrial enlargement.
Magnified view of the mitral valve in apical 4-chamber view revealing restricted opening of both leaflets.
Transesophageal echocardiogram in an apical 3-chamber view showing calcification and doming of the anterior mitral leaflet and restricted opening of both leaflets.
Transesophageal echocardiogram in an apical 3-chamber view with color Doppler interrogation of the mitral valve revealing aliasing, which is consistent with increased gradient across the mitral valve secondary to stenosis. Also shown in this image, a posteriorly directed jet of severe mitral regurgitation.
Table 1. Duration of Secondary Rheumatic Fever Prophylaxis
CategoryDuration After Last AttackRating*
Rheumatic fever with carditis and residual heart disease (persistent valvular disease† )10 y or until age 40 y (whichever is longer); sometimes lifelong prophylaxisIC
Rheumatic fever with carditis but no residual heart disease (no valvular disease† )10 y or until age 21 y (whichever is longer)IC
Rheumatic fever without carditis5 y or until age 21 y (whichever is longer)IC
*Rating indicates classification of recommendation and level of evidence (eg, IC indicates Class I, level of Evidence C).



†Clinical or echocardiographic evidence.



Table 2. Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)
AgentDoseModeRating*
Benzathine penicillin GChildren 27 kg (60 lb): 600,000 U



Patients >27 kg: 1,200,000 every 4 wk†



IntramuscularIA
Penicillin V250 mg bidOralIB
SulfadiazineChildren 27 kg: 0.5 g qd



Patients >27 kg: 1 g qd



OralIB
Macrolide or azalide (for individuals allergic to penicillin and sulfadiazine)VariableOralIC
*Rating indicates classification of recommendation and level of evidence (eg, IA indicates Class I, level of Evidence A).



†In high-risk situations, administration every 3 weeks is justified and recommended.



Table 3. Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis*)
AgentDoseModeDurationRating
Penicillins
Penicillin V (phenoxymethyl penicillin)Children 27 kg (60 lb): 250 mg bid or tid



Patients >27 kg: 500 mg bid or tid



Oral10 dIB
Amoxicillin50 mg/kg qd (maximum 1 g)Oral10 dIB
Benzathine penicillin GChildren 27 kg (60 lb): 600,000 U



Patients >27 kg: 1,200,000 U



IntramuscularOnceIB
For individuals allergic to penicillin
Narrow-spectrum cephalosporin (cephalexin, cefadroxil)VariableOral10 dIB
Clindamycin20 mg/kg/d divided in 3 doses (maximum 1.8 g/d)Oral10 dIIaB
Azithromycin12 mg/kg qd (maximum 500 mg)Oral5 dIIaB
Clarithromycin15 mg/kg/d divided bid (maximum 250 mg bid)Oral10 dIIaB
*Sulfonamides, trimethoprim, tetracyclines, and fluoroquinolones are not acceptable.



† Rating indicates classification of recommendation and level of evidence (eg, IB indicates Class I, level of Evidence B)



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