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Shortness of breath
in a 51 year old
woman

Sean Coffey FRACP
Gerard Wilkins FRACP
Bernard Prendergast FESC
Dunedin Hospital, New Zealand and
John Radcliffe Hospital, Oxford,
United Kingdom
Our patient – medical history and examination

•
•
•
•

51 year old woman
Progressive shortness of breath on exertion for 2 yrs
No angina, syncope or other cardiovascular symptoms
Hypertension for 6 years
• Beta-blocker for 5 years
Our patient – medical history and examination

•
•
•
•

No other significant past medical history
Sinus bradycardia 50 beats/minute
No murmurs noted
No other significant findings
Our patient - echocardiography

Watch
video

Parasternal long
axis - systole

Watch
video

Parasternal long
axis - diastole

Parasternal short
axis - diastole
What is your diagnosis?

• Myxomatous mitral valve?
• Mitral annular dilatation?
• Rheumatic mitral valve?
• Mitral valve endocarditis?
• Mitral annular calcification?
What is your diagnosis?

• Myxomatous mitral valve?
• Mitral annular dilatation?
• Rheumatic mitral valve
• Mitral valve endocarditis?
• Mitral annular calcification?
Morphological features of rheumatic mitral stenosis1

• Anterior mitral valve leaflet thickening ≥5 mm (for
those older than 40 years)
• Chordal thickening

• Restricted leaflet motion (e.g. due to commissural
fusion or chordal shortening)
• Excessive leaflet tip motion during systole (due to
elongation of the chords, resulting in regurgitation)

1.

World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease—an
evidence-based guideline. Reményi, B. et al. Nat. Rev. Cardiol. 9, 297–309 (2012).
Echo assessment of mitral stenosis1

• Degree and location of calcification
• Mitral valve area according to:
•
•
•

•
•
•
•

1.

2D planimetry (reference method)
Pressure half-time
(Continuity equation and proximal isovelocity surface area) level 2 recommendations

Mean transvalve gradient
Pulmonary artery pressure
Left atrial size
Presence or absence of rheumatic features on other
valves
Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice.
Baumgartner, H. et al. Eur J Echocardiogr 10, 1–25 (2009).
Echo assessment of mitral stenosis

• Suitability for percutaneous valvuloplasty can be assessed
by transthoracic echocardiography
• The Wilkins score1 is based on
•
•
•
•

leaflet mobility
leaflet thickening
calcification
subvalvular thickening

• The Cormier score2 is based on
• subvalvular thickening
• finding of any calcification on fluoroscopy

1.
2.

Percutaneous balloon dilatation of the mitral valve: an analysis of echocardiographic variables related to
outcome and the mechanism of dilatation. Wilkins, GT et al. Br Heart J 60, 299-308, (1988).
Evaluation by two-dimensional and Doppler echocardiography of the results of percutaneous mitral
valvuloplasty. Cormier, B. et al. Arch Mal Coeur Vaiss 82, 185-191, (1989).
Grading of severity of mitral stenosis1

Mild

Moderate

Severe

> 1.5

1.0 – 1.5

< 1.0

Mean gradient (mmHg)*

<5

5 – 10

> 10

Pulmonary artery pressure (mmHg)

< 30

30 – 50

> 50

Specific finding
Valve area (cm2)
Supportive findings

* Mean gradient applicable at heart rates between 60 and 80 bpm and in sinus rhythm

1.

Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice.
Baumgartner, H. et al. Eur J Echocardiogr 10, 1–25 (2009).
Our patient - mitral valve Doppler
E velocity: 1.59 m/s
Peak gradient: 10.1mmHg
Half maximum pressure
= 0.707 x maximum velocity
= 1.12 m/s

Pressure half time (PHT)
= time for pressure to decrease to
half maximum pressure
= 348 ms

Mitral valve area in cm2 (by PHT)
= 220 ms / PHT
= 0.64 cm2 i.e. severe mitral stenosis

NB: Pressure gradients are highly flow
dependent, and lower during long diastolic
intervals. Mean gradient would only
classify this as moderate mitral stenosis.
Additional imaging

• When is transoesophageal echo indicated?1
• To look for left atrial thrombus
• Before percutaneous mitral commissurotomy (PMC)
• After an embolic event
• Poor transthoracic images

• When is stress echo indicated?2
• Level 2 recommendation for
• Equivocal symptoms
• Symptoms out of proportion to degree of stenosis
1.
2.

Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J
33, 2451-2496, (2012).
Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice.
Baumgartner, H. et al. Eur J Echocardiogr 10, 1–25 (2009).
Our patient - transthoracic echo report

• Mitral valve area (planimetry) – 1.1 cm2
• Mitral valve area (PHT) – 0.64 cm2
• Mean gradient – 6 mmHg (but unreliable due to
bradycardia)
• Pulmonary artery pressure – 24mmHg + right atrial
pressure
• Left atrial 2D dimension – 5.3cm
• Left atrial volume index – 62 ml/m2
• Also
• No other affected valves
• Normal left ventricular systolic function
What is your management?

• Open surgical mitral commissurotomy?
• Percutaneous mitral commissurotomy?
• Conservative management?
• Anticoagulation with Factor Xa inhibitor?
• Anticoagulation with warfarin?
(choose as many options as required)
How should this woman be managed?

• Open surgical mitral commissurotomy?
• Percutaneous mitral commissurotomy
• Conservative management?
• Anticoagulation with Factor Xa inhibitor?
• Anticoagulation with warfarin
When is intervention indicated?1

• Valve area ≤ 1.5cm2 and
• Symptoms or
• In patients suitable for percutaneous mitral
commissurotomy (PMC)
• Symptoms produced by exercise testing or
• High risk of embolism or haemodynamic decompensation

• High embolic risk if
• Previous embolism, dense spontaneous contrast (SEC) in left atrium, or
recent or paroxysmal atrial fibrillation

• High risk of haemodynamic decompensation if
• Systolic pulmonary artery pressure > 50mmHg at rest, need for major
non-cardiac surgery, or desire for pregnancy
1.

Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J
33, 2451-2496, (2012).
Which intervention should be used?1
• PMC should be chosen unless there are
• Unfavourable anatomical characteristics
• Wilkins score > 8, Cormier score 3, very small mitral valve
area, severe tricuspid regurgitation
• Unfavourable clinical characteristics
• Old age, history of commissurotomy with persistent
commissural opening, NYHA class IV, permanent atrial
fibrillation, severe pulmonary hypertension

1.

Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J
33, 2451-2496, (2012).
Which intervention should be used?1

• Even if anatomy is unfavourable, PMC can be considered as
initial treatment if clinical characteristics are favourable.
• Contraindications to PMC
Left atrial thrombus
More than mild mitral regurgitation
Severe or bicommissural calcification
Absence of commissural fusion
Severe concomitant aortic valve disease, or severe combined tricuspid
stenosis and regurgitation
• Concomitant coronary artery disease requiring bypass surgery
•
•
•
•
•

1.

Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J
33, 2451-2496, (2012).
Our patient - decision making

• Anatomy was not favourable for PMC
• Reduced mobility of base and mid-leaflets
• Calcification extending into mid-leaflet
• Wilkins score 10, Cormier score 3

• But, favourable clinical characteristics
•
•
•
•
•

Relatively young age
No prior commissurotomy
NYHA II
No atrial fibrillation
No pulmonary hypertension
Our patient – PMC

Watch video

PMC was performed under local
anaesthetic, and using fluoroscopy and
echocardiographic guideance. A Mullins
sheath was advanced from the femoral
vein to the right atrium.

The interatrial septum was crossed with a
Brockenbrough needle. An Inoue wire
was delivered to the left atrium and
looped, allowing removal of the Mullins
sheath.
Our patient - PMC

The mitral valve was crossed and the
Inoue Balloon partially inflated to
expand the distal portion of the
balloon.

The balloon was then pulled back against
the mitral valve and fully inflated,
completing commisurotomy.
When is anticoagulation indicated?
• Mitral stenosis carries a high risk of
thromboembolism, even in sinus rhythm
• Anticoagulation is indicated1
• In patients with AF
• In patients in sinus rhythm with any of the following
features:
•
•
•
•

Previous history of thromboembolism
Thrombus visible in left atrium
Spontaneous echo contrast (SEC) in the left atrium
Severely dilated left atrium
• M-mode dimension > 5.0 cm or LA volume index > 60ml/m2

• Factor Xa and direct thrombin inhibitors are not
indicated for use in these settings
1.

Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J
33, 2451-2496, (2012).
Our patient - progress

• No complications following PMC
• Echo prior to discharge
• Mitral valve area (planimetry) 1.6cm2
• Mild mitral regurgitation unchanged

• Discharged from hospital the day after PMC
• Exercise capacity increased to previous levels at 1
year follow-up
What are the outcomes after PMC?
• In one large series1, 89% of patients had a good immediate
result after PMC, defined as mitral valve area ≥1.5 cm2 and
no more than mild MR
• Early serious adverse events were uncommon
• In-hospital death 0.4%
• Embolic stroke 0.3%
• Moderate or severe MR 3.4%

• 20 year results related to immediate results
• Poor immediate result: 5% good long term result (free at 20 years from
cardiovascular death, mitral intervention, or NYHA III or IV heart failure)
• Good immediate result: 33% good long term result

• Even in the group with good immediate results, valve area
and pressure gradient measured after PMC predict
outcomes 20 years later
1.

Late Results of Percutaneous Mitral Commissurotomy up to 20 Years. Development and Validation of a
Risk Score Predicting Late Functional Results From a Series of 912 Patients. Bouleti, C. et al. Circulation
125, 2119-2127, (2013).
Take home messages

• Patients with rheumatic heart disease may have no history
of rheumatic fever
• Severity of mitral stenosis is assessed primarily using mitral
valve area measured by planimetry and pressure half time
• Pressure gradients can be misleading in the setting of high
or low heart rates, and in patients with atrial fibrillation
• Percutaneous mitral commissurotomy should be considered
the first line treatment in patients with symptoms and
mitral valve area < 1.5 cm2
• Anticoagulation should be considered for patients with
mitral stenosis with high risk features for thromboembolism
(even if in sinus rhythm)
• Early results of percutaneous mitral commissurotomy
predict results up to 20 years later
Join the ESC Working Group
on Valvular Heart Disease
and take part in its
activities !

Membership is FREE!

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Shortness of breath in a 51 year old woman

  • 1. Shortness of breath in a 51 year old woman Sean Coffey FRACP Gerard Wilkins FRACP Bernard Prendergast FESC Dunedin Hospital, New Zealand and John Radcliffe Hospital, Oxford, United Kingdom
  • 2. Our patient – medical history and examination • • • • 51 year old woman Progressive shortness of breath on exertion for 2 yrs No angina, syncope or other cardiovascular symptoms Hypertension for 6 years • Beta-blocker for 5 years
  • 3. Our patient – medical history and examination • • • • No other significant past medical history Sinus bradycardia 50 beats/minute No murmurs noted No other significant findings
  • 4. Our patient - echocardiography Watch video Parasternal long axis - systole Watch video Parasternal long axis - diastole Parasternal short axis - diastole
  • 5. What is your diagnosis? • Myxomatous mitral valve? • Mitral annular dilatation? • Rheumatic mitral valve? • Mitral valve endocarditis? • Mitral annular calcification?
  • 6. What is your diagnosis? • Myxomatous mitral valve? • Mitral annular dilatation? • Rheumatic mitral valve • Mitral valve endocarditis? • Mitral annular calcification?
  • 7. Morphological features of rheumatic mitral stenosis1 • Anterior mitral valve leaflet thickening ≥5 mm (for those older than 40 years) • Chordal thickening • Restricted leaflet motion (e.g. due to commissural fusion or chordal shortening) • Excessive leaflet tip motion during systole (due to elongation of the chords, resulting in regurgitation) 1. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease—an evidence-based guideline. Reményi, B. et al. Nat. Rev. Cardiol. 9, 297–309 (2012).
  • 8. Echo assessment of mitral stenosis1 • Degree and location of calcification • Mitral valve area according to: • • • • • • • 1. 2D planimetry (reference method) Pressure half-time (Continuity equation and proximal isovelocity surface area) level 2 recommendations Mean transvalve gradient Pulmonary artery pressure Left atrial size Presence or absence of rheumatic features on other valves Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. Baumgartner, H. et al. Eur J Echocardiogr 10, 1–25 (2009).
  • 9. Echo assessment of mitral stenosis • Suitability for percutaneous valvuloplasty can be assessed by transthoracic echocardiography • The Wilkins score1 is based on • • • • leaflet mobility leaflet thickening calcification subvalvular thickening • The Cormier score2 is based on • subvalvular thickening • finding of any calcification on fluoroscopy 1. 2. Percutaneous balloon dilatation of the mitral valve: an analysis of echocardiographic variables related to outcome and the mechanism of dilatation. Wilkins, GT et al. Br Heart J 60, 299-308, (1988). Evaluation by two-dimensional and Doppler echocardiography of the results of percutaneous mitral valvuloplasty. Cormier, B. et al. Arch Mal Coeur Vaiss 82, 185-191, (1989).
  • 10. Grading of severity of mitral stenosis1 Mild Moderate Severe > 1.5 1.0 – 1.5 < 1.0 Mean gradient (mmHg)* <5 5 – 10 > 10 Pulmonary artery pressure (mmHg) < 30 30 – 50 > 50 Specific finding Valve area (cm2) Supportive findings * Mean gradient applicable at heart rates between 60 and 80 bpm and in sinus rhythm 1. Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. Baumgartner, H. et al. Eur J Echocardiogr 10, 1–25 (2009).
  • 11. Our patient - mitral valve Doppler E velocity: 1.59 m/s Peak gradient: 10.1mmHg Half maximum pressure = 0.707 x maximum velocity = 1.12 m/s Pressure half time (PHT) = time for pressure to decrease to half maximum pressure = 348 ms Mitral valve area in cm2 (by PHT) = 220 ms / PHT = 0.64 cm2 i.e. severe mitral stenosis NB: Pressure gradients are highly flow dependent, and lower during long diastolic intervals. Mean gradient would only classify this as moderate mitral stenosis.
  • 12. Additional imaging • When is transoesophageal echo indicated?1 • To look for left atrial thrombus • Before percutaneous mitral commissurotomy (PMC) • After an embolic event • Poor transthoracic images • When is stress echo indicated?2 • Level 2 recommendation for • Equivocal symptoms • Symptoms out of proportion to degree of stenosis 1. 2. Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J 33, 2451-2496, (2012). Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. Baumgartner, H. et al. Eur J Echocardiogr 10, 1–25 (2009).
  • 13. Our patient - transthoracic echo report • Mitral valve area (planimetry) – 1.1 cm2 • Mitral valve area (PHT) – 0.64 cm2 • Mean gradient – 6 mmHg (but unreliable due to bradycardia) • Pulmonary artery pressure – 24mmHg + right atrial pressure • Left atrial 2D dimension – 5.3cm • Left atrial volume index – 62 ml/m2 • Also • No other affected valves • Normal left ventricular systolic function
  • 14. What is your management? • Open surgical mitral commissurotomy? • Percutaneous mitral commissurotomy? • Conservative management? • Anticoagulation with Factor Xa inhibitor? • Anticoagulation with warfarin? (choose as many options as required)
  • 15. How should this woman be managed? • Open surgical mitral commissurotomy? • Percutaneous mitral commissurotomy • Conservative management? • Anticoagulation with Factor Xa inhibitor? • Anticoagulation with warfarin
  • 16. When is intervention indicated?1 • Valve area ≤ 1.5cm2 and • Symptoms or • In patients suitable for percutaneous mitral commissurotomy (PMC) • Symptoms produced by exercise testing or • High risk of embolism or haemodynamic decompensation • High embolic risk if • Previous embolism, dense spontaneous contrast (SEC) in left atrium, or recent or paroxysmal atrial fibrillation • High risk of haemodynamic decompensation if • Systolic pulmonary artery pressure > 50mmHg at rest, need for major non-cardiac surgery, or desire for pregnancy 1. Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J 33, 2451-2496, (2012).
  • 17. Which intervention should be used?1 • PMC should be chosen unless there are • Unfavourable anatomical characteristics • Wilkins score > 8, Cormier score 3, very small mitral valve area, severe tricuspid regurgitation • Unfavourable clinical characteristics • Old age, history of commissurotomy with persistent commissural opening, NYHA class IV, permanent atrial fibrillation, severe pulmonary hypertension 1. Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J 33, 2451-2496, (2012).
  • 18. Which intervention should be used?1 • Even if anatomy is unfavourable, PMC can be considered as initial treatment if clinical characteristics are favourable. • Contraindications to PMC Left atrial thrombus More than mild mitral regurgitation Severe or bicommissural calcification Absence of commissural fusion Severe concomitant aortic valve disease, or severe combined tricuspid stenosis and regurgitation • Concomitant coronary artery disease requiring bypass surgery • • • • • 1. Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J 33, 2451-2496, (2012).
  • 19. Our patient - decision making • Anatomy was not favourable for PMC • Reduced mobility of base and mid-leaflets • Calcification extending into mid-leaflet • Wilkins score 10, Cormier score 3 • But, favourable clinical characteristics • • • • • Relatively young age No prior commissurotomy NYHA II No atrial fibrillation No pulmonary hypertension
  • 20. Our patient – PMC Watch video PMC was performed under local anaesthetic, and using fluoroscopy and echocardiographic guideance. A Mullins sheath was advanced from the femoral vein to the right atrium. The interatrial septum was crossed with a Brockenbrough needle. An Inoue wire was delivered to the left atrium and looped, allowing removal of the Mullins sheath.
  • 21. Our patient - PMC The mitral valve was crossed and the Inoue Balloon partially inflated to expand the distal portion of the balloon. The balloon was then pulled back against the mitral valve and fully inflated, completing commisurotomy.
  • 22. When is anticoagulation indicated? • Mitral stenosis carries a high risk of thromboembolism, even in sinus rhythm • Anticoagulation is indicated1 • In patients with AF • In patients in sinus rhythm with any of the following features: • • • • Previous history of thromboembolism Thrombus visible in left atrium Spontaneous echo contrast (SEC) in the left atrium Severely dilated left atrium • M-mode dimension > 5.0 cm or LA volume index > 60ml/m2 • Factor Xa and direct thrombin inhibitors are not indicated for use in these settings 1. Guidelines on the management of valvular heart disease (version 2012). Vahanian, A. et al. Eur Heart J 33, 2451-2496, (2012).
  • 23. Our patient - progress • No complications following PMC • Echo prior to discharge • Mitral valve area (planimetry) 1.6cm2 • Mild mitral regurgitation unchanged • Discharged from hospital the day after PMC • Exercise capacity increased to previous levels at 1 year follow-up
  • 24. What are the outcomes after PMC? • In one large series1, 89% of patients had a good immediate result after PMC, defined as mitral valve area ≥1.5 cm2 and no more than mild MR • Early serious adverse events were uncommon • In-hospital death 0.4% • Embolic stroke 0.3% • Moderate or severe MR 3.4% • 20 year results related to immediate results • Poor immediate result: 5% good long term result (free at 20 years from cardiovascular death, mitral intervention, or NYHA III or IV heart failure) • Good immediate result: 33% good long term result • Even in the group with good immediate results, valve area and pressure gradient measured after PMC predict outcomes 20 years later 1. Late Results of Percutaneous Mitral Commissurotomy up to 20 Years. Development and Validation of a Risk Score Predicting Late Functional Results From a Series of 912 Patients. Bouleti, C. et al. Circulation 125, 2119-2127, (2013).
  • 25. Take home messages • Patients with rheumatic heart disease may have no history of rheumatic fever • Severity of mitral stenosis is assessed primarily using mitral valve area measured by planimetry and pressure half time • Pressure gradients can be misleading in the setting of high or low heart rates, and in patients with atrial fibrillation • Percutaneous mitral commissurotomy should be considered the first line treatment in patients with symptoms and mitral valve area < 1.5 cm2 • Anticoagulation should be considered for patients with mitral stenosis with high risk features for thromboembolism (even if in sinus rhythm) • Early results of percutaneous mitral commissurotomy predict results up to 20 years later
  • 26. Join the ESC Working Group on Valvular Heart Disease and take part in its activities ! Membership is FREE!