Mitral Stenosis: Evaluation and Management

Laura Immordino, MD February 10, 2010

Etiologies of MS

Rheumatic 99% Other:

Severe MAC with degeneration Congential Medications Carcinoid syndrome Connective tissue diseases Complications of prior MV surgery Radiation Injury

Severe MAC causing MS

Progresses from leaflet base tips Accelerated by:

Aging Hypertension CKD

Most often appreciated in posterior MV annulus Rarely progresses to the point of causing clinically relevant obstruction to flow

Congenital MS

Types of Congenital MS:

Anatomic abnormality of the valve/chordae that causes decreased mobility or a decreased orifice Parachute MV single pap muscle

Abnormal position of chordae to the posterior mitral leaflet, which restricts its motion, leading to mitral stenosis

Parachute MV

Typically occurs with a single pap muscle to which all chordae of an otherwise normal valve attach leaflet mobility restricted restriction of MV inflow

Medications causing MS

Fenfluramine (Fen-phen) has been linked to mitral stenosis

Distinctive valvular abnormalities seen on surgical removal, with plaque-like process on leaflets surfaces and encasing the chordae, similar to valve damage by carcinoid tumors

Carcinoid syndrome causing MS

Carcinoid tumors arise from the GI tract

Malignant carcinoid syndrome occurs when serotonin and other vasoactive substances are released from carcinoid tumor cells More than of patients will develop carcinoid heart disease as a late complication (after hepatic metastases)

Most common heart involvement is valvular disease Generally affects right heart, since vasoactive hormones are inactivated by monoamine oxidase in the lungs

Left-sided involvement can be seen with intracardiac shunts, extensive bronchial mets, or high tumor secretions that overwhelm the metabolic capacity of the lungs Usually causes combination of MS and MR with diffuse valve thickening

Connective Tissue Diseases

Systemic lupus erythematosus Rheumatoid arthritis Ehlers-Danlos WeillMarchesani syndrome (rare CTD) Mucopolysaccharide diseases (HunterHurler)

Thickened mitral valve apparatus in a patient with Weill-Marchesani syndrome.

Complications of Prior MV Surgery/Repair

Rare as complication of MVR

In kids, may need to upsize when they reach adolescence

Can occur as a complication of MVRe

Alfieri stitch to repair severe MR from MVP

Radiation-induced Injury

Typically occurs 10 15 years after exposure Generally affects anterior leaflet more extensively Most common findings: fibrosis & stiffening of proximal portions of the anterior leaflet

Pathologic echodensity of anterior leaflet with reduced mobility. Also increased echodensities in the AV.

Rheumatic MS

Exact etiology debated

Chronic autoimmune process?

Cross-reactivity between M protein of Group A Strep and valve tissue
8 rheumatogenic serotypes, Pharyngeal infection required (not seen in impetigo)

Prevalence and age of symptomatic MS varies geographically

North America/Europe:

Africa:

Prevalence= 1/100,000 Symptomatic MS usually occurs at 50-60 yo Prevalence= 35/100,000 Symptomatic MS often seen in teenagers

Rate of rheumatic fever is roughly equal among genders, MS is 2-3 times more common in women than men. Generally takes 10-20 yrs to develop post-exposure

?Continuing low-grade rheumatic process leading to valve destruction, versus hemodynamic stresses on a now-injured valve

Rheumatic MS

Continuous, progressive, lifelong disease

Slow, stable course in early years Progressively accelerates later in life

Once symptoms develops, generally about a decade before they become disabling

Mortality

10-yr survival of untreated pts presenting with MS: 50-60%

If asymptomatic/minimally symptomatic: > 80% If symptoms are limiting: 0-15% If severe pulmonary hypertension: 3-yr mean survival Progressive pulmonary and systemic congestion (60-70%) Systemic embolism (20-30%) PE (10%) Infection (1-5%)

Mortality due to:

Rheumatic Heart Disease

Valvular effects

Isolated MS 25% of patients 40% have MS + MR > 1/3 have multivalve involvement
AV >> TV Rarely affects PV

Also causes atrial inflammation

Combo of atrial inflammation + increased LAP from MS LAE, fibrosis of atrial wall and disorganization of LA muscle bundles AF

Rheumatic MS Echo Findings

Thickening of leaflet tips

Tips bases

diastolic doming

Symmetric fusion of commissures = small central oval orifice Chordal shortening and fusion

If exclusively fusion/contraction predominately MR

Differential Diagnosis

LA outflow obstruction from:

Myxoma Ball-valve thrombus in LA Infective endocarditis with large vegetation Congenital membrane in LA (cor triatriatum) Congenital supravalvular stenosing ring

LA myxoma

LA thrombus

Cor Triatriatum

Fibrous membrane effectively partitions the LA into 2 chambers Associated anomalies:

ASD Persistent left SVC

Supravalvular Stenosing Ring

Fibrosing membranes closer to MV, may adhere to leaflets How to differentiate from rheumatic MS:

Absence of anterior leaflet doming Color Doppler shows flow acceleration and turbulence at level of annulus, rather than leaflet tips

Also associated with ASD, persistent left SVC

Clinical Presentation

Dyspnea = most common

Pulmonary edema attacks may be initiated by effort, emotional stress, fever, AF, pregnancy (anything that flow) Bronchial vein rupture

Hemoptysis (rare)

Pts may develop PV-bronchial vein shunts

Pulmonary infarct from chronic pulmonary hypertension Pink frothy sputum from acute pulmonary edema

Chest pain not typical Palpitations from rapid AF Hoarseness Ortner syndrome

Compression of left recurrent laryngeal nerve by dilated LA/PA

Right-sided heart failure

Physical Exam Findings

Most common: irregular pulse, signs of L/R heart failure Other findings:

Diastolic murmur @ apex

Other causes: MAC (90% of elderly pts with diastolic rumble will be due to MAC), severe MR, LA myxoma, HCM (early diastolic flow into hypertrophied, non-distendable LV)

Loud S1 (when leaflets are flexible) as calcification of leaflets progresses get S1 Prominent a wave on jugular venous exam if in SR RV heave Loud/widely transmitted P2 Right-sided S4 S3 is notably absent (unless significant MR/AI co-exists) Opening Snap (0.04 0.12 s after A2)

Time interval varies inversely with LAP (MV opens earlier with severe MS and high LAP)

Definition of MS

Normal MVA = 4.0 5.0 cm2 MVA:

1.5 2.0 = mild MS 1.0 1.5 = moderate MS < 1.0 = severe MS

Requires transmitral PG of ~ 20mm Hg to maintain normal CO at rest so LAP typically > 25mm Hg

Echo Findings

M-mode first clinically used to detect MS

Diminished diastolic E-F slope of anterior leaflet

Correlates poorly with degree of stenosis not used in quantification Also seen in AV disease (AI/AS) due to LV filling rate from LV compliance

Concordant motion of anterior and posterior leaflets

Diastolic anterior motion of the posterior leaflet

Thickened leaflets with decreased motion, increased reflectivity

Echo Findings

2D Findings

Thickened, calcified leaflets Diastolic doming (rheumatic MS) particularly of anterior leaflet

Hockey-stick appearance

Symmetric commissural fusion (fish mouth)

Echo Findings

Determination of MS severity:

Planimetry PHT Flow Area Continuity Equation PISA Mean/peak PG Pulmonary pressures

Planimetry

- With adequate imaging, most accurate method of assessing MVA - Measure at level of leaflet tips (otherwise will overestimate MVA)

Pressure Half Time

Use CW through MV

Time for LA-LV PG to decrease by Decel time measured on E wave

PHT = Decel Time x 0.29 MVA = 220/PHT Severe AI or high filling pressures shortens PHT, which overestimates MVA Immediately s/p PBMV Abnormalities of LA or LV compliance

Used to calculate MVA

Less reliable with:

Pressure Half Time

Flow Area

Using Color Doppler

Width of diastolic jet measured in A3 and A4 chamber views Used to calculate MVA
MVA = (0.785)(Diameter#1)(Diameter#2)

Continuity Equation

Flow through MV = Flow through AV

A1V1 = A2 V2 A1 = A2 V2 / V1

A1 = (0.785)(DLVOT2)(VTILVOT) /(VTIMV inflow) = MVA

LVOT measured in parasternal long VTILVOT measured in A3 or A5 (PW) VTIMV inflow measured in A4 (CW) If significant AI, can compare flow through MV annulus against flow through MV tips

Only accurate if AV is competent

PISA

When to use:

When PHT affected by AI, 2-D planimetry images unsuitable for evaluation Irregular rhythms
Color Doppler while zoomed on MV in A4 Aliasing velocity moved towards LV Measure distance from point of aliasing to MV orifice Measure angle of MV leaflets MVA = [(6.28)(PISA r2)(Va)/ (MS peak v)] x (ao/180o)

How to calculate PISA:

Used to calculate MVA

Pressure Gradients

Using CW Doppler through MV in apical views, measure TVI

P = 4v2 Peak and mean PG calculated

Both elevated with MS Mean PG correlates well with MS severity Peak PG also elevated in other conditions do not use to calculate MS severity

Echo Findings

Quantification of MS
*applicable when HR is 60 90 bpm

Mild
Mean gradient (mmHg)

Moderate 5 10

Severe > 10

<5

PASP (mmHg)
Valve area (cm2)

< 30
> 1.5

30 50
1.0 1.5

>50
< 1.0

Hemodynamics of MS

First bouts of dyspnea in MS pts are usually precipitated by tachycardia (exercise, pregnancy, anemia, infxn, AF)

P = 4v2

flow = LAP

HR = diastolic filling time

Combination of LAP + filling time explains why previously asx patients with MS can suddenly develop SOB + pulmonary edema with the onset of rapid AF

Loss of atrial contraction = CO by ~ 20% as well

Complications of MS

Most common LV abnormalities:

Underfilled LV Paradoxical septal motion from RV pressure overload

pts with isolated MS have EF

Most likely due to chronically preload + afterload

?extension of scarring from MV to adjacent myocardium vs. co-existing ICM

Regional HK has been described

LV compliance may be seen due to leftward displacement of IV septum (from more rapid early filling of RV)

Complications of MS

LAE + blood stasis = thrombus formation

PAH

Rate of embolic stroke in pt with MS + AF = 7-15%/year

Is a result of:

Can result in cor pulmonale

Passive transmission of LAP Reactive pulmonary arteriolar constriction (from pulmonary venous hypertension) Obliterative changes in pulmonary vascular bed

RV fxn usually maintained with moderately PASP (3060mm Hg) When PASP > 60 mm Hg:

RV will initially fail to exhibit hyperkinesis with exercise Ultimately will develop RV dysfxn, dilation at rest

Management of MS

Symptomatic vs Asymptomatic

If asymptomatic check exercise stress > or < 1.5 cm2

MVA

Assessment of valve morphology to determine if favorable for PMBV

Wilkins Score > or < 50 mm Hg

Mild/mod vs Severe Pulmonary Hypertension

+/- AF

Wilkins Score
Grade Mobility
Highly mobile, only tips restricted Mid-base of leaflets have normal mobility Diastolic motion, mainly at base

Subvalvular Thickening
Minimal thickening, just below leaflets Chordal thickening, up to 1/3 of length Thickening to distal 1/3 of chords

Thickening
Near normal thickness (4-5 mm)

Calcification
Single area of increased brightness

1 2 3 4

Thickening of Scattered margins (5-8 areas of mm), midbrightness in leaflets normal leaflet margins Thickening through entire leaflet (5-8 mm) Considerable thickening of entire leaflet (8-10 mm) Brightness extends to midportion of leaflet Extensive brightness throughout most of leaflet

No/minimal forward Extensive movement in thickening, diastole shortening of all chordal structures

Wilkins Score

Management Strategy for Patients With Mitral Stenosis

Bonow, R. O. et al. J Am Coll Cardiol 2008;52:e1-e142

*Surveillence echos are not recommended, unless there is a change in clinical status, or patient has severe MS Copyright 2008 American College of Cardiology Foundation. Restrictions may apply.

Management Strategy for Patients With Mitral Stenosis and Moderate to Severe Symptoms

Bonow, R. O. et al. J Am Coll Cardiol 2008;52:e1-e142

Copyright 2008 American College of Cardiology Foundation. Restrictions may apply.

Management Strategy for Patients With Mitral Stenosis and Mild Symptoms

Bonow, R. O. et al. J Am Coll Cardiol 2008;52:e1-e142

Copyright 2008 American College of Cardiology Foundation. Restrictions may apply.

Medical Management of MS

Asx, mild MS no specific tx Mod-severe MS

Avoidance of physical stressors to avoid tachycardia (flow + diastolic filling)

Encourage pt to pursue low-level aerobic exercise Recommendations for exercise are usually symptom-limited

Bbl/CCB may benefit pts in SR with exertional symptoms Na restriction/prn diuretics to avoid pulmonary edema May try rhythm-control in select pts Rate control, anticoagulation = mainstay of therapy

Recurrent paroxysmal AF

Medical Management of MS

Risk of systemic embolization is 7-15%/year in patients with MS

Risk Factors = age, AF Does not appear to relate to MS severity, CO, LA size or CHF symptoms

ACC/AHA Guidelines for Prevention of Systemic Embolization

Pts with MS and AF Class Ib Asx pts in SR with sev MS and LAE ( 5.5cm) +/- SEC Class IIb/c Goal INR 2.5 3.5

Surgical Management of MS

Mitral commissurotomy

First performed in 1920s Open commissurotomy and MVR were surgical procedures of choice for MS

Surgical risk: 1-3% 5-yr complication-free survival: 80-90% 5-yr re-operation rate: 4-7%

Open preferred over closed, allows for direct inspection and commissural divison, splitting of fused chordae and pap muscles and debridement of calcium deposits under direct vision LAA amputation is recommended during procedure If MV apparatus markedly deformed, can decide to do MVR at time of surgery

Surgical Management of MS

PMBV emerged in 1980s, clinically approved in 1994

One or more large balloons inflated across MV using a catheterbased approach Immediate results similar to commissurotomy

Initially, double-balloon technique was used Now, hourglass-shaped single balloon used (Inoue balloon)
MVA usually doubles (1.0 2.0 cm2) 50-60% decrease in transmitral PG Overall, 80-95% successful (MVA > 1.5 and LAP < 18) Severe MR (2-10%) Residual ASD

Complications:

Mortality rate: 1-2% Event-free survival: 50-65% over 3-7 years

LV perforation (0.5-4%) Embolic events (0.5-3%) MI (0.3-0.5%)

Large ASD ( > 1.5:1) in < 12% with double, < 5% with Inoue

80-90% in patients with favorable valve morphology

PMBV

Surgical Management of MS

Contraindications to PBMV:

Significant MR (moderate-severe) Persistent LA thrombus despite adequate anticoagulation TEE performed to look for LA or LAA thrombus

Before PBMV is performed:

If +, anticoagulated for several weeks/months, then repeat TEE performed If thrombus persists despite adequate anticoagulation, surgical commissurotomy/valve replacement is indicated