Mitral stenosis resident survival guide

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Twinkle Singh, M.B.B.S. [2]; Mohamed Moubarak, M.D. [3]; Amr Marawan, M.D. [4]; Rim Halaby, M.D. [5]

Mitral Stenosis Resident Survival Guide Microchapters
Overview
Causes
Stages
FIRE
Diagnosis
Treatment
Medical Therapy
Intervention
Secondary Prevention of Rheumatic Fever
Do's

Overview

Mitral stenosis refers to abnormal narrowing of mitral orifice, which leads to obstruction of blood flow from left atrium to left ventricle most commonly as a complication of rheumatic fever. The most common presentations of mitral stenosis are dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and peripheral edema. Mitral stenosis has a characteristic low-pitched, rumbling diastolic murmur, heard best at the apex during physical examination. The definitive therapy for mitral stenosis include percutaneous balloon valvotomy, surgical mitral valve repair, or mitral valve replacement.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Click here for the complete list of causes

Stages

Shown below is a table depicting the 4 stages of mitral stenosis, adapted from 2014 AHA/ACC guidelines for management of valvular heart diseases.[2]

Abbreviations: MS: mitral stenosis; MVA: mitral valve area; PASP: pulmonary artery systolic pressure

Stage Definition Valve anatomy Valve hemodynamics Hemodynamic consequences Symptoms
A At risk of MS ❑ Mild diastolic doming of mitral valve leaflets ❑ Normal transmitral velocity Absent Absent
B Progressive MS ❑ Rheumatic valve changes characterized by commissural fusion and diastolic doming of mitral valve
❑ MVA > 1.5 cm2 (planimetered)
❑ Increased transmitral flow velocities
❑ MVA > 1.5 cm2
❑ Pressure half time during diastole < 150 ms
❑ Mild to moderate left atrial enlargement
❑ Normal pulmonary pressure at rest
None
C Asymptomatic severe MS ❑ Rheumatic valve changes characterized by commissural fusion and diastolic doming of mitral valve
❑ MVA ≤ 1.5 cm2
❑ MVA ≤ 1 cm2 in very severe MS
❑ MVA ≤ 1.5 cm2 (planimetered)
❑ MVA ≤ 1 cm2 (planimetered) in very severe MS
❑ Diastolic pressure half time ≥ 150 ms
❑ Diastolic pressure half time ≥ 220 ms with very severe MS
❑ Severe left atrial enlargement
❑ PASP > 30 mm Hg
Absent
D Symptomatic severe MS ❑ Rheumatic valve changes characterized by commissural fusion and diastolic doming of mitral valve
❑ MVA ≤ 1.5 cm2 (planimetered)
❑ MVA ≤ 1.5 cm2
❑ MVA ≤ 1 cm2 in very severe MS
❑ Diastolic pressure half time ≥ 150 ms
❑ Diastolic pressure half time ≥ 220 ms with very severe MS
❑ Severe left atrial enlargement
❑ PASP > 30 mm Hg
Dyspnea on exertion
❑ Decreased exercise tolerance

FIRE: Focused Initial Rapid Evaluation

A Focused Initial Rapid Evaluation (FIRE) should be performed to identify patients in need of immediate intervention.

Boxes in the red color signify that an urgent management is needed.

 
 
 
 
 
 
Identify cardinal findings that increase the pretest probability of mitral stenosis

Mid diastolic murmur
❑ Low-pitched diastolic rumble
❑ Associated with an opening snap
❑ Best heard at the cardiac apex
❑ Radiating to the axilla
❑ Increases with lying down, raising the legs and with exercise
❑ Decreases with valsalva maneuver and amyl nitrate
❑ Reduced pulse pressure
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient have any of the following findings of decompensated mitral stenosis that require urgent management?
Tachycardia
Hypotension
Severe dyspnea
Loss of consciousness
Chest pain
❑ Sudden weakness or paralysis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
What is the complication of mitral stenosis that is causing decompensation?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

❑ Suspect in case of:
❑ Sudden weakness or paralysis - face, arm or leg
❑ Speech or visual difficulties
Altered level of consciousness
❑ Sudden severe headache
 

❑ Suspect in case of palpitations
❑ Order an ECG immediately looking for
❑ Irregularly irregular rhythm, and
❑ Absent P waves
 

❑ Suspect in case of:
❑ Acute onset of exertional dyspnea or dyspnea at rest
❑ Pleuritic or substernal chest pain
Hemoptysis
 

❑ Suspect in case of severe dyspnea
❑ Increased jugular venous pressure immediately

Hepatomegaly ± pulsatile liver

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
❑ Treat the complications of mitral stenosis that lead to decompensation
❑ Order a TTE to evaluate the severity of the mitral stenosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
When to consider intervention in mitral stenosis ?
Continue with the treatment algorithm below
 
 
 
 
 
 
 
 

Complete Diagnostic Approach

A complete diagnostic approach should be carried out after a focused initial rapid evaluation is conducted and following initiation of any urgent intervention.[2].
Abbreviations: AF: Atrial fibrillation; PMBC: Percutaneous mitral ballon commissurotomy; TR: Tricuspid regurgitation; S1: First heart sound; P2: Pulmonary component of second heart sound; EKG: Electrocardiogram; TTE: Transthoracic echocardiography; MS: Mitral stenosis

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Inquire about past medical history:
Rheumatic fever
Respiratory infection
❑ Congenital MS
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Examine the patient:

Appearance of the patient
❑ Plethoric cheeks with bluish patches

Vital signs

Pulse

❑ Rate
Tachycardia
❑ Rhythm
Irregularly irregular (suggestive of AF)
❑ Strength
❑ Reduced pulse pressure
❑ Reduced in volume

Neck:
Jugular venous distension

❑ Prominent a wave (suggestive of right heart failure)
❑ Absent a wave (suggestive of AF)
❑ Prominent v wave (suggestive of TR)

Extremities:
Peripheral edema (suggestive of right sided heart failure)

Abdominal examination:
Ascites (suggestive of right sided heart failure)
Hepatomegaly (suggestive of right sided heart failure)

Cardiovascular examination:

Auscultation
❑ Left parasternal heave
❑ Loud S1
❑ Loud P2 (indicates pulmonary hypertension)
❑ Opening snap
Murmur

❑ Most prominent at the apex and best heard using the bell of the stethoscope
Mid diastolic murmur (low pitched, rumbling)
Holosystolic murmur (suggestive of TR)
Graham-Steell murmur (suggestive of pulmonary regurgitation)

{{#ev:youtube|HW2pk1icYdM|250}}
Video adapted from Youtube.com

Rales
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Order tests:

Transthoracic echocardiography

❑ Assess valve area
❑ Assess disease of other valves
❑ Assess mean pressure gradient
❑ Assess pulmonary artery pressure
❑ Assess suitability of valve morphology for PMBC

❑ Exercise testing with Doppler or invasive hemodynamic assessment in case of discrepancy between resting Doppler echocardiographic findings and clinical symptoms or signs

❑ Assess the response of the mean mitral gradient
❑ Assess the pulmonary artery pressure

EKG

Left atrial enlargement
❑ Broad, bifid P wave in lead II (P mitrale)


Picture adapted from en.ecgpedia.org

❑ Biphasic P wave with terminal negative portion

Left atrial enlargement as seen in lead V1
Picture adapted from en.ecgpedia.org

Right ventricular hypertrophy
Right axis deviation of +90 degrees or more
❑ Incomplete RBBB pattern
❑ ST T strain pattern in leads 2,3,aVF
P pulmonale or right atrial enlargement or P congenitale
❑ Inverted T wave in the anterior precordial leads

Click here for the rest of the EKG findings in right ventricular hypertrophy

Right axis deviation
QRS complex is positive in leads III and aVF
QRS complex is negative in leads I and aVL

Shown below an ECG depicting right axis deviation

Atrial fibrillation
❑ Absence of P waves
❑ Irregularly irregular heart rate

Shown below an ECG depicting atrial fibrillation

Picture adapted from Wikidoc.org

Chest X-ray

❑ Double right heart border (suggestive of left atrial hypertrophy)
❑ Prominent pulmonary artery
Kerley lines (suggestive of interstitial pulmonary edema)


Picture adapted from Radiopedia.org

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Classify mitral stenosis based on the following findings on TTE:
❑ Valve anatomy
❑ Valve hemodynamics gradient
❑ Hemodynamic consequences
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Treatment

Medical Therapy

Treatment of Acute Decompensation
❑ Digoxin
In the following cases:

❑ Symptomatic right ventricular or left ventricular dysfunction (digitalis increases myocardial contractility)
Atrial fibrillation (digitalis slows the ventricular response which prolongs the diastolic filling time)[3]

❑ Diuretics (In the following cases fluid overload)
❑ Low sodium diet
❑ Activity restriction


Systemic Embolization Prevention[2]
In the following cases:
❑ Paroxysmal, persistent, or permanent atrial fibrillation (Class I, Level of Evidence B)
❑ Prior embolization event (Class I, Level of Evidence B)
Left atrial thrombus (Class I, Level of Evidence B)


Rate Control[2]
In the following cases:
Atrial fibrillation associated with fast ventricular response (Class IIa, Level of Evidence C)

❑ Normal sinus rhythm plus symptoms associated with exercise (Class IIb, Level of Evidence B)
 

Intervention

Shown below is an algorithm depicting the indications for mitral stenosis intervention according to the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease.[2]

Abbreviations: AF: atrial fibrillation; MR: mitral regurgitation; MS: mitral stenosis; MVA: mitral valve area ; PCWP: pulmonary capillary wedge pressure; PMBC: percutaneous mitral balloon commissurotomy; T1/2: pressure half-time

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Determine the severity of rheumatic mitral stenosis (MS)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Very severe MS
❑ MVA ≤1 cm2
❑ T1/2 ≥220 ms
 
 
 
 
 
 
 
Severe MS
❑ MVA ≤1.5 cm2
❑ T1/2 ≥150 ms
 
 
 
 
 
 
 
 
 
 
Progressive MS
MVA >1.5 cm2
T1/2 <150 ms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Is the patient symptomatic?
 
 
 
 
 
 
 
Is the patient symptomatic?
 
 
 
 
 
 
 
 
 
 
Is the patient symptomatic?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
( Stage C)
 
 
 
 
 
 
Yes
(Stage D)
 
 
 
 
 
 
 
 
 
 
No
(Stage C)
 
 
 
Yes
With no other cause for the symptoms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient have:
❑ Favorable valve morphology
AND
❑ No left atrial clot
AND
❑ No or mild MR
 
 
 
 
 
 
Does the patient have:
❑ Favorable valve morphology
AND
❑ No left atrial clot
AND
❑ No or mild MR
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Is there a new onset of AF?
 
 
 
Order an exercise treadmill test
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
 
Yes
 
Yes
 
 
 
No
 
 
 
 
 
Yes
 
No
 
Is PCWP> 25 mm Hg?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient have:
NYHA class III-IV symptoms
AND
❑ High surgical risk
 
 
 
 
 
Does the patient have:
❑ Favorable valve morphology
AND
❑ No left atrial clot
AND
❑ No or mild MR
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
 
Yes
 
Yes
 
No
 
 
Yes
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Periodic monitoring
 
PMBC (Class IIa)
 
PMBC (Class I)
 
Mitral valve surgery (Class I)
 
 
 
PMBC (Class IIb)
 
 
 
 
Periodic monitoring
 
PMBC (Class IIb)
 
Periodic monitoring

Secondary Prevention of Rheumatic Fever

Indications

Shown below is the table depicting the indication for secondary prophylaxis of rheumatic fever.[4]

Indications Duration of prophylaxis
Rheumatic fever with carditis and persistent valvular heart disease 10 years or until the patient is 40 years (whichever is longer) (Class I, Level of Evidence C)
Rheumatic fever with carditis but no valvular heart disease 10 years or until the patient is 21 years (whichever is longer) (Class I, Level of Evidence C)
Rheumatic fever without carditis 5 years or until the patient is 21 years (whichever is longer) (Class I, Level of Evidence C)

Antibiotic Regimens

Shown below is the table depicting the antibiotic regimens for secondary prophylaxis of rheumatic fever.[4]

Antibiotics Dosage
Penicillin G benzathine
(Class I, Level of Evidence A)
Weight >27 Kg (60 lb): 1.2 million units IM every day for 4 weeks
Weight ≤27 Kg (60 lb): 600,000 units IM every day for 4 weeks
Penicillin V
(Class I, Level of Evidence B)
200 mg orally twice a day
Sulfadiazine
(Class I, Level of Evidence B)
Weight >27 Kg (60 lb): 1 g orally once a day
Weight ≤27 Kg (60 lb): o.5 g orally once a day
Macrolide or azalide antibiotics (in patients allergic to penicillin)
(Class I, Level of Evidence C)
Varies

Do's

References

  1. Tadele, H.; Mekonnen, W.; Tefera, E. (2013). "Rheumatic mitral stenosis in Children: more accelerated course in sub-Saharan Patients". BMC Cardiovasc Disord. 13 (1): 95. doi:10.1186/1471-2261-13-95. PMID 24180350. Unknown parameter |month= ignored (help)
  2. 2.0 2.1 2.2 2.3 2.4 Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA; et al. (2014). "2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines". Circulation. doi:10.1161/CIR.0000000000000029. PMID 24589852.
  3. Boon NA, Bloomfield P (2002). "The medical management of valvar heart disease". Heart. 87 (4): 395–400. PMC 1767079. PMID 11907022.
  4. 4.0 4.1 Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST; et al. (2009). "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. 119 (11): 1541–51. doi:10.1161/CIRCULATIONAHA.109.191959. PMID 19246689.


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