eMedicine Specialties > Cardiology > Valvular Heart Disease
Mitral Stenosis: Treatment & Medication
Updated: Nov 9, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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Treatment
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).
- The current American Heart Association (AHA) recommendations4 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.
[#Table1]Table 1. Duration of Secondary Rheumatic Fever Prophylaxis
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Table
| Category | Duration After Last Attack | Rating* |
| Rheumatic fever with carditis and residual heart disease (persistent valvular disease† ) | 10 y or until age 40 y (whichever is longer); sometimes lifelong prophylaxis | IC |
| 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 carditis | 5 y or until age 21 y (whichever is longer) | IC |
| Category | Duration After Last Attack | Rating* |
| Rheumatic fever with carditis and residual heart disease (persistent valvular disease† ) | 10 y or until age 40 y (whichever is longer); sometimes lifelong prophylaxis | IC |
| 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 carditis | 5 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)
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Table
| Agent | Dose | Mode | Rating* |
| Benzathine penicillin G | Children 27 kg (60 lb): 600,000 U Patients >27 kg: 1,200,000 every 4 wk† | Intramuscular | IA |
| Penicillin V | 250 mg bid | Oral | IB |
| Sulfadiazine | Children 27 kg: 0.5 g qd Patients >27 kg: 1 g qd | Oral | IB |
| Macrolide or azalide (for individuals allergic to penicillin and sulfadiazine) | Variable | Oral | IC |
| Agent | Dose | Mode | Rating* |
| Benzathine penicillin G | Children 27 kg (60 lb): 600,000 U Patients >27 kg: 1,200,000 every 4 wk† | Intramuscular | IA |
| Penicillin V | 250 mg bid | Oral | IB |
| Sulfadiazine | Children 27 kg: 0.5 g qd Patients >27 kg: 1 g qd | Oral | IB |
| Macrolide or azalide (for individuals allergic to penicillin and sulfadiazine) | Variable | Oral | IC |
*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.
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 ³ 50 mm Hg at rest or ³ 60 mm Hg with exercise, or pulmonary capillary wedge pressure is ³ 25 mm Hg with exercise.5
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
- 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.
Consultations
A cardiology and/or cardiothoracic surgery consult may be necessary.
Diet
The patient should start a low-salt diet if pulmonary vascular congestion is present.
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.
Medication
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Antiarrhythmics
These agents alter the electrophysiologic mechanisms responsible for arrhythmia.
Digoxin (Lanoxicaps, Lanoxin)
Cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Adult
0.125-0.375 mg PO qd
Pediatric
Not established
IV calcium may produce arrhythmias in digitalized patients
Medications that may increase levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil
Medications that may decrease serum levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, and procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Documented hypersensitivity; beriberi heart disease, hypertrophic obstructive cardiomyopathy, constrictive pericarditis, and carotid sinus syndrome
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypokalemia may reduce positive inotropic effect; hypercalcemia predisposes patient to digitalis toxicity; hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis; adjust dose in renal impairment; highly toxic (overdoses can be fatal)
Amiodarone (Cordarone, Pacerone)
May inhibit AV conduction and sinus node function. Prolongs action potential and refractory period in myocardium and inhibits adrenergic stimulation. Prior to administration, control ventricular rate and CHF (if present) with digoxin or calcium channel blockers.
Adult
Loading dose: 800-1600 mg/d PO in 1-2 doses for 1-3 wk; decrease to 600-800 mg/d in 1-2 doses for 1 mo
Maintenance dose: 400 mg/d PO
Alternatively: 150 mg (10 mL) IV over first 10 min, followed by 360 mg (200 mL) over next 6 h, then 540 mg over next 18 h
Pediatric
Not established
Increases effect and blood levels of theophylline, quinidine, procainamide, phenytoin, methotrexate, flecainide, digoxin, cyclosporine, beta-blockers, and anticoagulants; cardiotoxicity is increased by ritonavir, sparfloxacin, and disopyramide; coadministration with calcium channel blockers may cause additive effect and further decrease myocardial contractility; cimetidine may increase levels; protease inhibitors (eg, indinavir, ritonavir, amprenavir, nelfinavir) inhibit metabolism, resulting in increased serum levels, and may prolong QT interval
Documented hypersensitivity; complete AV block; intraventricular conduction defects; patients taking ritonavir or sparfloxacin
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in thyroid or liver disease
Calcium channel blockers
In specialized conducting and automatic cells in the heart, calcium is involved in the generation of the action potential. Calcium channel blockers inhibit movement of calcium ions across the cell membrane, depressing both impulse formation (automaticity) and conduction velocity.
Diltiazem (Cardizem CD, Dilacor, Tiazac, Cardizem LA)
During depolarization, inhibits calcium ions from entering slow channels and voltage-sensitive areas of vascular smooth muscle and myocardium.
Adult
Cardizem SR: 60-120 mg PO bid
Cardizem CD: 180-240 mg PO qd in hypertension
Pediatric
Not established
May increase carbamazepine, digoxin, cyclosporine, and theophylline levels; when administered with amiodarone, may cause bradycardia and a decrease in cardiac output; when given with beta-blockers, may increase cardiac depression; cimetidine may increase levels
Documented hypersensitivity; severe CHF; sick sinus syndrome; second- or third-degree AV block; hypotension (<90 mm Hg systolic)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in impaired renal or hepatic function; may increase LFT levels, and hepatic injury may occur
Anticoagulants
These agents prevent recurrent or ongoing thromboembolic occlusion of the vertebrobasilar circulation.
Warfarin (Coumadin)
Interferes with hepatic synthesis of vitamin K–dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders. Tailor dose to maintain an INR of 2-3.
Adult
5-15 mg/d PO qd for 2-5 d; adjust dose according to desired INR
Pediatric
Not established
Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate
Medications that may increase anticoagulant effects include oral antibiotics, capecitabine, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac
Documented hypersensitivity; severe liver or kidney disease; open wounds or GI ulcers
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients with protein C or S deficiency are at risk of developing skin necrosis
Heparin
Augments activity of antithrombin III and prevents conversion of fibrinogen to fibrin. Does not actively lyse but is able to inhibit further thrombogenesis. Prevents reaccumulation of clot after spontaneous fibrinolysis.
Adult
Initial dose: 40-170 U/kg IV
Maintenance infusion: 18 U/kg/h IV
Alternatively: 50 U/kg/h IV initially, followed by continuous infusion of 15-25 U/kg/h; increase dose by 5 U/kg/h q4h prn using aPTT results
Pediatric
Not established
Digoxin, nicotine, tetracycline, and antihistamines may decrease effects; NSAIDs, aspirin, dextran, dipyridamole, and hydroxychloroquine may increase heparin toxicity
Documented hypersensitivity; subacute bacterial endocarditis; active bleeding; history of heparin-induced thrombocytopenia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
In neonates, preservative-free heparin is recommended to avoid possible toxicity (gasping syndrome) from benzyl alcohol, which is used as a preservative; caution in severe hypotension and shock; monitor for bleeding in peptic ulcer disease, menstruation, increased capillary permeability, and when giving IM injections
Beta-adrenergic blockers
These agents inhibit chronotropic, inotropic, and vasodilatory responses to beta-adrenergic stimulation.
Metoprolol (Lopressor, Toprol XL)
Selective beta1-adrenergic receptor blocker that decreases automaticity of contractions. During IV administration, carefully monitor blood pressure, heart rate, and ECG.
Adult
100 mg/d PO qd or divided bid/tid initially; increase at 1-wk intervals prn, not to exceed total of 450 mg/d
Pediatric
Not established
Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels, possibly resulting in decreased pharmacologic effects; toxicity may increase with coadministration of sparfloxacin, phenothiazines, astemizole, calcium channel blockers, quinidine, flecainide, and contraceptives; may increase toxicity of digoxin, flecainide, clonidine, epinephrine, nifedipine, prazosin, verapamil, and lidocaine
Documented hypersensitivity; uncompensated congestive heart failure; bradycardia, asthma; cardiogenic shock; AV conduction abnormalities
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second or third trimester; beta-adrenergic blockade may reduce signs and symptoms of acute hypoglycemia and may decrease clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; monitor patient closely and withdraw drug slowly; during IV administration, carefully monitor blood pressure, heart rate, and ECG
Antibiotics
Must cover all likely pathogens in the context of this clinical setting. Use as prophylaxis against streptococcal infections.
Penicillin G benzathine (Bicillin L-A, Permapen)
Interferes with synthesis of cell wall mucopeptides during active multiplication, which results in bactericidal activity. Used to treat syphilis and for prophylaxis of recurrent streptococcal infections.
Adult
2 million U IM qmo
Pediatric
Not established
Probenecid can increase effectiveness by decreasing clearance; coadministration with tetracyclines can decrease effectiveness
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in impaired renal function
Diuretics
Diuretics are used for treatment of pulmonary congestion. Treatment may improve symptoms of venous congestion through elimination of retained fluid and preload reduction.
Furosemide (Lasix)
Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. Dose must be individualized to patient. Depending on response, administer at increments of 20-40 mg, no sooner than 6-8 h after previous dose, until desired diuresis occurs. When treating infants, titrate with increments of 1 mg/kg/dose until a satisfactory effect is achieved.
Adult
20-80 mg/d PO/IV/IM; titrate up to 600 mg/d for severe edematous states
Pediatric
Not established
Metformin decreases concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently; increased plasma lithium levels and toxicity are possible when taken concurrently
Documented hypersensitivity; hepatic coma; anuria; state of severe electrolyte depletion
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform frequent serum electrolyte, carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
More on Mitral Stenosis |
| Overview: Mitral Stenosis |
| Differential Diagnoses & Workup: Mitral Stenosis |
 Treatment & Medication: Mitral Stenosis |
| Follow-up: Mitral Stenosis |
| Multimedia: Mitral Stenosis |
| References |
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References
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].
Bruce CJ, Nishimura RA. Newer advances in the diagnosis and treatment of mitral stenosis. Curr Probl Cardiol. Mar 1998;23(3):125-92. [Medline].
[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].
[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].
Feldman T. Rheumatic Mitral Stenosis. Curr Treat Options Cardiovasc Med. Apr 2000;2(2):93-104. [Medline].
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].
[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].
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.
Carabello BA. Modern management of mitral stenosis. Circulation. Jul 19 2005;112(3):432-7. [Medline].
Further Reading
Keywords
mitral stenosis, mitral valve stenosis, MVS, chronic rheumatic heart disease, congenital mitral stenosis, systemic lupus erythematosus, SLE, rheumatoid arthritis, RA, metabolism disorder, congenital metabolic disorder, metabolic disorder, Fabry's disease, Fabry disease, Hurler-Scheie syndrome, valve calcification, mitral valve calcification, infective endocarditis, carcinoid syndrome, acute rheumatic fever, ARF, congestive heart failure, CHF, heart disease, cardiac disease, amyloid deposition, amyloid, tricuspid regurgitation, hemoptysis
