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Mitral Stenosis Clinical Presentation

  • Author: Claudia Dima, MD, FACC; Chief Editor: Richard A Lange, MD, MBA  more...
 
Updated: Nov 06, 2014
 

History

Symptoms of mitral stenosis usually manifest during the third or fourth decade of life and nearly half of the patients do not recall a history of acute rheumatic fever.

Patients are generally asymptomatic at rest during the early stage of the disease. However, factors that increase heart rate such as fever, severe anemia, thyrotoxicosis, exercise, excitement, pregnancy, and atrial fibrillation may result in dyspnea.

Nearly 15% of patients develop embolic episodes that are usually associated with atrial fibrillation. Rarely, embolic episodes may occur even in the patient with sinus rhythm. Systemic embolization may lead to stroke, renal failure, or myocardial infarction.

Hoarseness can develop from compression of the left recurrent laryngeal nerve against the pulmonary artery by the enlarged left atrium. Also, compression of bronchi by the enlarged left atrium can cause persistent cough.

Hemoptysis may occur and is usually not fatal.

Pregnant women with mild mitral stenosis may become symptomatic during their second trimester because of the increase in blood volume and cardiac output.

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Physical

The presence of mitral facies (pinkish-purple patches on the cheeks) indicate chronic severe mitral stenosis leading to reduced cardiac output and vasoconstriction.

Jugular vein distension may be seen. In the patient with sinus rhythm, a prominent a wave reflects increased right atrial pressure from pulmonary hypertension and right ventricular failure. A prominent v wave is seen with tricuspid regurgitation.The apical impulse may be laterally displaced or not palpable, especially in cases of severe mitral stenosis. This can be explained by decreased left ventricular filling. Rarely, a diastolic thrill can be felt at the apex with the patient in the left lateral recumbent position. See the videos below.

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.
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.

Often a right ventricular lift is palpable in the left parasternal region in the patient with pulmonary hypertension. A P2 may be palpable in the 2nd left intercostal space.

The auscultatory findings characteristic of mitral stenosis are a loud first heart sound, an opening snap, and a diastolic rumble.

The first heart sound is accentuated because of a wide closing excursion of the mitral leaflets. The degree of loudness of the first heart sound depends on the pliability of the mitral valve. The intensity of the first heart sound diminishes as the valve becomes more fibrotic, calcified, and thickened.

The second heart sound is normally split, and the pulmonic component is accentuated if pulmonary hypertension is present. The opening snap follows the second heart sound. The sudden tensing of the valve leaflets after they have completed their opening excursion causes an opening snap. In patients with elevated left atrial pressure and hence with severe mitral stenosis, the opening snap occurs closer to the second heart sound.

The diastolic murmur of mitral stenosis is of low pitch, rumbling in character, and best heard at the apex with the patient in the left lateral position. It commences after the opening snap of the mitral valve, and the duration of the murmur correlates with the severity of the stenosis. The murmur is accentuated by exercise, whereas it decreases with rest and Valsalva maneuver. In patients with sinus rhythm, the murmur increases in intensity during late diastole (so called, presystolic accentuation) due to increased flow across the stenotic mitral valve caused by atrial contraction.

A high-pitched decrescendo diastolic murmur secondary to pulmonary regurgitation (Graham Steell murmur) may be audible at the upper sternal border.

A pansystolic murmur of TR and an S3 originating from the right ventricle may be audible in the 4th left intercostal space in the patient with right ventricular dilatation.

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Contributor Information and Disclosures
Author

Claudia Dima, MD, FACC Interventional Cardiology

Disclosure: Nothing to disclose.

Coauthor(s)

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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

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

Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD, MBA President, Texas Tech University Health Sciences Center, Dean, Paul L Foster School of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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

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 Institute, American College of Physicians, American Heart Association, American Medical Association

Disclosure: Received honoraria from Boehringer-Ingelheim for speaking and teaching.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous authors Holger P Salazar, MD, Senthil Nachimuthu, MD, FACP, and Kiruthika Balasundaram, MBBS, to the development and writing of this article.

References
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  2. 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. 1994 Feb 1. 120(3):177-83. [Medline].

  3. Henri C, Pierard LA, Lancellotti P, Mongeon FP, Pibarot P, Basmadjian AJ. Exercise Testing and Stress Imaging in Valvular Heart Disease. Can J Cardiol. 2014 Sep. 30(9):1012-1026. [Medline].

  4. Wunderlich NC, Beigel R, Siegel RJ. Management of mitral stenosis using 2D and 3D echo-Doppler imaging. JACC Cardiovasc Imaging. 2013 Nov. 6(11):1191-205. [Medline].

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

  6. Schlosshan D, Aggarwal G, Mathur G, Allan R, Cranney G. Real-time 3D transesophageal echocardiography for the evaluation of rheumatic mitral stenosis. JACC Cardiovasc Imaging. 2011 Jun. 4(6):580-8. [Medline].

  7. [Guideline] Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline 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. J Am Coll Cardiol. 2014 Jun 10. 63(22):e57-185. [Medline]. [Full Text].

  8. [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. 2009 Mar 24. 119(11):1541-51. [Medline].

  9. 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. 2010 May 18. 121(19):2130-6. [Medline].

  10. [Guideline] Nishimura RA, Carabello BA, Faxon DP, et al. 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. 2008 Aug 19. 52(8):676-85. [Medline].

  11. [Guideline] Vahanian A, Alfieri O, Andreotti F, et al. Guidelines on the management of valvular heart disease (version 2012): The Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2012 Oct. 33(19):2451-96. [Medline].

  12. Wann LS, Curtis AB, Ellenbogen KA, et al. 2011 ACCF/AHA/HRS focused update on the management of patients with atrial fibrillation (update on dabigatran): a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines. J Am Coll Cardiol. 2011 Mar 15. 57(11):1330-7. [Medline].

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



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



Patients >27 kg: 500 mg bid or tid



Oral 10 d IB
Amoxicillin 50 mg/kg qd (maximum 1 g) Oral 10 d IB
Benzathine penicillin G Children 27 kg (60 lb): 600,000 U



Patients >27 kg: 1,200,000 U



Intramuscular Once IB
For individuals allergic to penicillin
Narrow-spectrum cephalosporin (cephalexin, cefadroxil) Variable Oral 10 d IB
Clindamycin 20 mg/kg/d divided in 3 doses (maximum 1.8 g/d) Oral 10 d IIaB
Azithromycin 12 mg/kg qd (maximum 500 mg) Oral 5 d IIaB
Clarithromycin 15 mg/kg/d divided bid (maximum 250 mg bid) Oral 10 d IIaB
*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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