Occurs in Survivors of Hodgkin’s Disease Who Have Previously Undergone Radiation Therapy (see Hodgkins Disease, [[Hodgkins Disease]]): mitral stenosis occurs 10-20 yrs after the radiation therapy
Increasing Frequency in North American Elderly Population
Physiology
Mitral Annular Calcification with Extension of the Calcification into the Leaflets: causes both a narrowing of the annulus and rigidity of the leaflets without commissural fusion
Chronically Elevated Pulmonary Venous Pressure Results in Pulmonary Arterial Hypertension Via Various Mechanisms
Passive Increase in Pulmonary Artery Pressure
Pulmonary Vasoconstriction: may be mediated by the vasoconstrictor endothelin-1
Pathologic Pulmonary Vascular Remodeling: may be mediated by the vasoconstrictor endothelin-1
Small Pulmonary Arterial Intimal Hyperplasia, Distal Extension of Smooth Muscle, Medial Hypertrophy and Fibrosis, and Adventitial Hypertrophy
Small Pulmonary Venous Thickened, Fibrotic Media and “Arterialization”
Engorged Pulmonary Capillaries
Dilated, Thickened Pulmonary Lymphatics
Bronchopulmonary Anastomoses
Reversibility of Pulmonary Arterial Hypertension: pulmonary hypertension in the setting of mitral stenosis is potentially completely reversible (NEJM, 1994) [MEDLINE]
“Hockey-Stick” Deformity of Anterior Mitral Leaflet (Parasternal Long Axis View): characteristic of rheumatic mitral stenosis
M-Mode
Restricted Opening of Mitral Valve (Parasternal Long Axis View, Parasternal Short Axis View)
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) (Circulation, 2014) [MEDLINE]
General Recommendations
Transthoracic Echocardiogram is Recommended in the Initial Evaluation of Patients with Known or Suspected Valvular Heart Disease to Confirm the Diagnosis, Establish Etiology, Determine Severity, Assess Hemodynamic Consequences, Determine Prognosis, and Evaluate for Timing of Intervention (Class I Recommendation, Level of Evidence: B)
Transthoracic Echocardiogram is Recommended in Patients with Known Valvular Heart Disease with Any Change in Symptoms or Physical Examination Findings (Class I Recommendation, Level of Evidence: C)
Periodic Monitoring with Transthoracic Echocardiogram is Recommended in Asymptomatic Patients with Known Valvular Heart Disease at Intervals Depending on the Valvular Lesion, Severity, Ventricular Size, and Ventricular Function (Class I Recommendation, Level of Evidence: C)
Rheumatic Mitral Stenosis
TTE is Indicated in Patients with Signs or Symptoms of Mitral Stenosis to Establish the Diagnosis, Quantify Hemodynamic Severity (Mean Pressure Gradient, Mitral Valve Area, and Pulmonary Artery Pressure), Assess Concomitant Valvular Lesions, and Demonstrate Valve Morphology (to Determine Suitability for Mitral Commissurotomy) (Class I Recommendation, Level of Evidence B)
TEE Should Be Performed in Patients Considered for Percutaneous Mitral Balloon Commissurotomy to Assess the Presence or Absence of Left Atrial Thrombus and to Further Evaluate the Severity of Mitral Regurgitation (Class I Recommendation, Level of Evidence B)
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) [MEDLINE]
General Recommendations
Exercise Testing is Reasonable in Selected Patients with Asymptomatic Severe Valvular Heart Disease to Confirm the Absence of Symptoms, to Assess the Hemodynamic Response to Exercise, or to Determine Prognosis (Class IIa Recommendation, Level of Evidence: B)
Rheumatic Mitral Stenosis
Exercise Testing with Doppler or Invasive Hemodynamic Assessment is Recommended to Evaluate the Response of the Mean Mitral Gradient and Pulmonary Artery Pressure in Patients with Mitral Stenosis When There is a Discrepancy Between Resting Doppler Echocardiographic Findings and Clinical Symptoms or Signs (Class I Recommendation, Level of Evidence: C)
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) (Circulation, 2014) [MEDLINE]
General Recommendations
Cardiac Catheterization for Hemodynamic Assessment is Recommended in Symptomatic Patients When Noninvasive Tests are Inconclusive or When There is a Discrepancy Between the Findings on Noninvasive Testing and Physical Examination Regarding the Severity of the Valvular Lesion (Class I Recommendation, Level of Evidence: C)
DLCO: increased during episodes of diffuse alveolar hemorrhage
Clinical Manifestations
General Comments
Precipitants of Symptoms in Mitral Stenosis
Conditions Which Increase Cardiac Output (Which Increases the Transmitral Flow) or Cause Tachycardia (Which Decreases Diastolic Filling Time) Result in an Increased Trans-Mitral Pressure Gradient
Low-Pitched Diastolic Rumble Most Prominent at the Apex
Best Heard with Patient in the Left Lateral Position with Patient in Expiration
Best Heard with Bell of the Stethoscope: due to low frequency
Duration of the Murmur (But Not the Intensity of the Murmur) Correlates with the Severity of the Mitral Stenosis (i.e. Severity of the Trans-Mitral Gradient with Duration of Blood Flow Across the Mitral Valve)
Mild Mitral Stenosis: gradient is confined to atrial systole, resulting in the murmur being heard late in diastole, just before S1
Moderate Mitral Stenosis: gradient is present at the onset of the diastolic flow period, resulting in the murmur being heard immediately following the opening snap (this early diastolic murmur is decrescendo, becoming softer as the left atrial pressure falls and the trans-mitral gradient decreases)
Severe Mitral Stenosis: continuous gradient throughout all of the diastolic period from mitral valve opening to mitral valve closure
Very Severe Mitral Stenosis: mitral stenosis murmur may become inaudible or absent (due to very slow flow across the mitral valve)
Maneuvers
Inspiration -> A2-opening snap interval widens and a distinct P2 may be heard
Expiration -> murmur and opening snap are accentuated
Increasing Venous Return (Leg Lift) or Exercise -> enhances the gradient and murmur lengthens (while the A2-opening snap interval shortens)
Decreasing Venous Return (Amyl Nitrate, Valsalva Maneuver, Standing After Squatting) -> decreases the gradient and murmur shortens (while the A2-opening snap interval widens)
Normal Apical Impulse: usually
However, Apical Impulse May Be Decreased Due to Reduced Left Ventricular Filling
Prominent “a” Wave (Atrial Contraction) in Jugular Venous Pulsation
Note: the “a” wave is Absent in Patients with Atrial Fibrillation
Prominent “c-v” Wave (Tricuspid Regurgitation): when tricuspid regurgitation is present
Right Ventricular Heave and Accentuated P2: when pulmonary hypertension is present
Infective Endocarditis (see Endocarditis, [[Endocarditis]])
Chronically Elevated Pulmonary Venous Pressure Results in Pulmonary Arterial Hypertension Via Various Mechanisms
Passive Increase in Pulmonary Artery Pressure
Pulmonary Vasoconstriction: may be mediated by the vasoconstrictor endothelin-1
Pathologic Pulmonary Vascular Remodeling: may be mediated by the vasoconstrictor endothelin-1
Small Pulmonary Arterial Intimal Hyperplasia, Distal Extension of Smooth Muscle, Medial Hypertrophy and Fibrosis, and Adventitial Hypertrophy
Small Pulmonary Venous Thickened, Fibrotic Media and “Arterialization”
Engorged Pulmonary Capillaries
Dilated, Thickened Pulmonary Lymphatics
Bronchopulmonary Anastomoses
Reversibility of Pulmonary Arterial Hypertension: pulmonary hypertension in the setting of mitral stenosis is potentially completely reversible (NEJM, 1994) [MEDLINE]
Systemic Embolization
Epidemiology: embolization is a common presentation of mitral stenosis
Sites of Systemic Embolization
Cerebral: most common site
Kidneys
Spleen
Coronary Artery: resulting in myocardial infarction
Physiology
Most Emboli Originate from the Left Atrium
Emboli May Originate from the Right Atrium in Some Cases: due to pulmonary hypertension with associated right atrial and ventricular dilatation
Associated with Emboli Originating from the Right Side of the Heart
Other Manifestations
Absence of Renal or Other Systemic Involvement
Secondary Prevention of Rheumatic Fever (see Rheumatic Fever, [[Rheumatic Fever]])
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) (Circulation, 2014) [MEDLINE]
Secondary Prevention of Rheumatic Fever is Indicated in Patients with Rheumatic Heart Disease, Specifically Mitral Stenosis (Class I Recommendation, Level of Evidence: C)
Agents
Penicillin G benzathine: 1.2 million units IM every 3-4 wk
Penicillin V potassium: 250 mg orally BID
Sulfadiazine: 1 g orally once daily
Macrolide or Azalide antibiotic: for patients allergic to penicillin and sulfadiazine
Duration of Prophylaxis
Rheumatic fever with carditis and residual heart disease (persistent VHD): prophylaxis given for 10 y or until patient is 40 y of age (whichever is longer)
Rheumatic fever with carditis but no residual heart disease (no valvular disease): prophylaxis given for 10 y or until patient is 21 y of age (whichever is longer)
Rheumatic fever without carditis: prophylaxis given for 5 y or until patient is 21 y of age (whichever is longer)
Treatment
General Management
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) (Circulation, 2014) [MEDLINE]
Site of Evaluation for Rheumatic Mitral Stenosis
Patients with Severe Valvular Heart Disease Should Be Evaluated by a Multidisciplinary Heart Valve Team When Intervention is Considered (Class I Recommendation, Level of Evidence: C)
Consultation with or Referral to a Heart Valve Center of Excellence is Reasonable When Discussing Treatment Options for Asymptomatic Patients with Severe Valvular Heart Disease, Patients Who May Benefit from Valve Repair vs Valve Replacement, or Patients with Multiple Comorbidities for Whom Valve Intervention is Considered (Class IIa Recommendation, Level of Evidence: C)
Infective Endocarditis Prophylaxis (see Endocarditis, [[Endocarditis]])
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) (Circulation, 2014) [MEDLINE]
Prophylaxis Against Infective Endocarditis Before Dental Procedures Which Involve Manipulation of Gingival Tissue, Manipulation of the Periapical Region of the Teeth, or Perforation of the Oral Mucosa is Reasonable for the Following Patients at the Highest Risk for Adverse Outcomes from Infective Endocarditis (Class IIa Recommendation, Level of Evidence B)
Patient with Prosthetic Cardiac Valve
Patient with History of Infective Endocarditis
Cardiac Transplant Recipients with Valvular Regurgitation Due to a Structurally Abnormal Valve
Patient with Congenital Heart Disease with the Following
Unrepaired Cyanotic Congenital Heart Disease (Including Palliative Shunts and Conduits)
Completely Repaired Congenital Heart Defect Repaired with Prosthetic Material or Device (Whether Placed by Surgery or Catheter Intervention) During the First 6 mo After the Procedure
Repaired Congenital Heart Disease with Residual Defects at the Site or Adjacent to the Site of a Prosthetic Patch or Prosthetic Device
Prophylaxis Against Infective Endocarditis is Not Recommended for Non-Dental Procedures (TEE, Esophagogastroduodenoscopy, Colonoscopy, or Cystoscopy) in the Absence of Active Infection in Patients with Valvular Heart Disease Who are at Risk of Infective Endocarditis (Class III Recommendation, Level of Evidence B)
Anticoagulation
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease) (Circulation, 2014) [MEDLINE]
Rheumatic Mitral Stenosis
Indications for Anticoagulation (Heparin or Coumadin) (Level of Evidence: B)
MS and AF (paroxysmal, persistent, or permanent)
MS and a prior embolic event
MS and a left atrial thrombus
Heart Rate Control
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease) (Circulation, 2014) [MEDLINE]
Rheumatic Mitral Stenosis
Heart rate control may be considered for patients with MS in normal sinus rhythm and symptoms associated with exercise (Class IIb Recommendation, Level of Evidence: B)
It is well known that the proportion of the cardiac cycle occupied by diastole decreases with increasing heart rate, thereby increasing the mean flow rate across the mitral valve (assuming constant cardiac output) with a consequent rise in mean mitral gradient in MS in proportion to the square of the flow rate
Heart rate control can be beneficial in patients with MS and AF and fast ventricular response (Class IIa Recommendation, Level of Evidence: C)
Heart rate control may be considered for patients with MS in normal sinus rhythm and symptoms associated with exercise (Class IIb Recommendation, Level of Evidence: C)
Management of Congestive Heart Failure (CHF) (see Congestive Heart Failure, [[Congestive Heart Failure]])
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Percutaneous Mitral Valve Balloon Commissurotomy (see xxxx, [[xxxx]])
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) (Circulation, 2014) [MEDLINE]
Rheumatic Mitral Stenosis
TEE should be performed in patients considered for percutaneous mitral balloon commissurotomy to assess the presence or absence of left atrial thrombus and to further evaluate the severity of MR (Class I Recommendation, Level of Evidence: B)
Percutaneous mitral balloon commissurotomy is recommended for symptomatic patients with severe MS (mitral valve area 1.5 cm2, stage D) and favorable valve morphology in the absence of left atrial thrombus or moderate-severe MR (Class I Recommendation, Level of Evidence: A)
Mitral valve surgery (repair, commissurotomy, or valve replacement) is indicated in severely symptomatic patients (NYHA class III to IV) with severe MS (mitral valve area 1.5 cm2, stage D) who are not high risk for surgery and who are not candidates for or who have failed previous percutaneous mitral balloon commissurotomy (Class I Recommendation, Level of Evidence: B)
Percutaneous Mitral Balloon Commissurotomy is Reasonable for Asymptomatic Patients with Very Severe Mitral Stenosis (Mitral Valve Area ≤1.0 cm2, Stage C) and Favorable Valve Morphology in the Absence of Left Atrial Thrombus or Moderate-Severe Mitral Regurgitation (Class IIa Recommendation, Level of Evidence C)
Percutaneous Mitral Balloon Commissurotomy May Be Considered for Asymptomatic Patients with Severe Mitral Stenosis (Mitral Valve Area ≤1.5 cm2, Stage C) and Valve Morphology Favorable for Percutaneous Mitral Balloon Commissurotomy in the Absence of Left Atrial Thrombus or Moderate-Severe Mitral Regurgitation Who Have New Onset of Atrial Fibrillation (Class IIb Recommendation, Level of Evidence C)
Percutaneous Mitral Balloon Commissurotomy May Be Considered for Symptomatic Patients with Mitral Valve Area >1.5 cm2 if There is Evidence of Hemodynamically Significant Mitral Stenosis Based on Pulmonary Artery Wedge Pressure >25 mm Hg or Mean Mitral Valve Gradient >15 mm Hg During Exercise (Class IIb Recommendation, Level of Evidence C)
Percutaneous Mitral Balloon Commissurotomy May Be Considered for Severely Symptomatic Patients (NYHA class III to IV) with Severe Mitral Stenosis (Mitral valve Area ≤1.5 cm2, Stage D) Who Have a Suboptimal Valve Anatomy and Who are Not Candidates for Surgery or at High Risk for Surgery (Class IIb Recommendation, Level of Evidence C)
Pulmonary Hypertension Usually Improves Over a Period of Months Postoperatively
Clinical Efficacy
Retrospective Review of Patients with Mitral Stenosis and Severe Pulmonary Hypertension (PA-Systolic ≥60 mm Hg or PA-Mean ≥50 mm Hg) Who Underwent Cardiac Surgery (Circulation, 1995) [MEDLINE]
Perioperative Mortality Rate: 11.6%
Predictors of Perioperative Death
Acute Presentation
Clinical Evidence of Right Ventricular Failure
Impaired Left Ventricular Ejection Fraction
Increased Left Ventricular Diastolic Pressure
Predictors of Complications
Acute Presentation
EKG Evidence of Right Ventricular Hypertrophy
Elevated Right Ventricular Systolic Pressure
Only Predictor of Long-Term Mortality: advanced age
Prognosis
Pulmonary Hypertension Increases the Perioperative Risk in Patients Having Mitral Valve Replacement
Case Series of Mitral Valve Replacement Examining the Impact of Pulmonary Hypertension (Ann Thorac Cardiovasc Surg, 2013) [MEDLINE]
Mitral Valve Replacement is Safe in the Presence of Pulmonary Hypertension: pulmonary pressures decreased over the first 24 hrs post-op
Recommendations (American Heart Association/American College of Cardiology Guidelines for Valvular Heart Disease, 2014) (Circulation, 2014) [MEDLINE]
Rheumatic Mitral Stenosis
Mitral Valve Surgery (Repair, Commissurotomy, or Valve Replacement) is Indicated in Severely Symptomatic Patients (NYHA Class III to IV) with Severe Mitral Stenosis (Mitral Valve Area ≤1.5 cm2, Stage D) Who are Not High Risk for Surgery and Who are Not Candidates for or Who Have Failed Previous Percutaneous Mitral Balloon Commissurotomy (Class I Recommendation, Level of Evidence B)
Concomitant Mitral Valve Surgery is Indicated for Patients with Severe Mitral Stenosis (Mitral Valve Area ≤1.5 cm2, Stage C or D) Undergoing Cardiac Surgery for Other Indications (Class I Recommendation, Level of Evidence C)
Mitral Valve Surgery is Reasonable for Severely Symptomatic Patients (NYHA class III to IV) with Severe Mitral Stenosis (Mitral Valve Area ≤1.5 cm2, Stage D), Provided There are Other Operative Indications (Aortic Valve Disease, CAD, TR, Aortic Aneurysm) (Class IIa Recommendation, Level of Evidence C)
Concomitant Mitral Valve Surgery May Be Considered for Patients with Moderate Mitral Stenosis (Mitral Valve Area 1.6 cm2 to 2.0 cm2) Undergoing Cardiac Surgery for Other Indications (Class IIb Recommendation, Level of Evidence C)
Mitral Valve Surgery and Excision of the Left Atrial Appendage May Be Considered for Patients with Severe Mitral Stenosis (Mitral Valve Area ≤1.5 cm2, stages C and D) Who Have Had Recurrent Embolic Events While Receiving Adequate Anticoagulation (Class IIb Recommendation, Level of Evidence C)
References
General
Percutaneous balloon valvuloplasty compared with open surgical commissurotomy for mitral stenosis. N Engl J Med. 1994;331(15):961 [MEDLINE]
Long-term outcome of cardiac surgery in patients with mitral stenosis and severe pulmonary hypertension. Circulation. 1995 Nov 1;92(9 Suppl):II137-42 [MEDLINE]
Early hemodynamic changes after mitral valve replacement in patients with severe and mild pulmonary artery hypertension. Ann Thorac Cardiovasc Surg. 2013;19(3):201-6. Epub 2012 Oct 15 [MEDLINE]
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. Circulation. 2014 Jun 10;129(23):e521-643. doi: 10.1161/CIR.0000000000000031. Epub 2014 Mar 3 [MEDLINE]
Diagnosis
Higgins CB, Reinke RT, Jones NE, Broderick T. Left atrial dimension on the frontal thoracic radiograph: a method for assessing left atrial enlargement. AJR Am J Roentgenol. 1978;130(2):251-255 [MEDLINE]
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 Thorac Cardiovasc Surg. 2014 Jul;148(1):e1-e132. doi: 10.1016/j.jtcvs.2014.05.014. Epub 2014 May 9 [MEDLINE]
