• Admitted on 1/26/2011
• 80 Y female
– weakness and nausea x 1 day.
– Had troponin leak
Ruled out for ACS
Started CPAP and Nocturnal oxygen
Cardiology consulted for Troponin leak
Planned for RHC & LHC
– Not done due to high INR
– Then again for candidiasis in groin area.
Past Medical History
• RA (rheumatoid arthritis)
• Atrial fibrillation
• Right Diaphragm paralysis with right middle lobe
collapse. 2008 ? probably had since birth. admission
6/08 for hypoxia, multifactorial sectondary to right
phrenic nerve palsy. On home oxygen therapy 2L NC
• OSA with hypoventilation, does not use CPAP
• DVT (deep venous thrombosis) 5/08
• Aortic stenosis
Unspecified essential hypertension
BCC (basal cell carcinoma of skin)
RA (refractory anemia)
• Mother: HTN, CAD (died at 94)
• Father: melanoma
• Social history:
– 5 pack years, quit 1972
– No EtOH
– Retired homemaker
• ClonazePAM (KLONOPIN) 0.5 mg tablet Take 0.5 mg by mouth nightly.
• Escitalopram (LEXAPRO) 20 MG tablet Take 1 Tab by mouth
• Lisinopril (PRINIVIL OR ZESTRIL) 40 MG tablet Take 1 Tab by
• Metoprolol (LOPRESSOR) 25 MG tablet Take 1 Tab by mouth 2
times daily. pt needs f/u visit for further refills.
• Potassium chloride SA 20 MEQ tablet Take 20 mEq by
• Warfarin (COUMADIN) 3 MG tablet Take 6 mg by mouth every
Monday. Take 3 mg by mouth every Sunday, Tuesday, Wednesday,
Thursday, Friday & Saturday.
ECHO done 1/27/2011
LV size is upper normal. There is severe asymmetric basal septal hypertrophy, with
an approximate 2:1 septal to inferolateral wall ratio.
There also appears to be systolic anterior motion of the mitral chords.
There is at least a 40 mm Hg peak LVOT dynamic gradient, which is probably
underestimated due to technical limitation of study.
Visually estimated LVEF is 65-70%.
The right ventricle appears moderately enlarged with normal systolic function.
The left atrium visually appears severely enlarged.
The right atrium is severely enlarged.
There are moderate fibrocalcific changes of the aortic valve which does not
appear to be significantly stenotic.Mild to moderate aortic regurgitation.
The mitral leaflets appear mildly thickened. There is moderate mitral annular
calcification present.There is mild to moderate mitral regurgitation.
There is mild to moderate tricuspid regurgitation.
Estimated PA systolic pressure by tricuspid regurgitant Doppler velocity is 60
No obvious significant pericardial effusion.
• On 1/30/2011 – increasing oxygen requirements
& found unresponsive.
• She was found to be in Atrial fibrillation
• ABG shows – respiratory acidosis & hypoxia.
• Transferred to MICU
• Acute hypercapnic respiratory failure,
superimposed on chronic respiratory
• Acute neurologic failure (no drugs)
• Atrial fibrillation with RVR
• Urinary retention
• Hypertrophic cardiomyopathy with SAM
• SIRS possible sepsis (fever of 101 Of)
• She was intubated A/C 18/600/5/70
• Started on amiodarone drip
• Started on Norepinephrine which was
weaned off in 5 hours.
• CVP 8-13
• Started on Vancomycin + Zosyn
• Given IVF ~ 2L
• Antibiotics changed as per culture reports
– Urine grew enterococcus
– Sputum miniBAL grew MRSA
• Extubated on 2/1/2011
• BiPAP at night after extubation
• Beta blockers added: metoprolol
increased to 150mg BID to control HR
• Was the hypotensive crisis due to atrial
fibrillation with some role of sepsis?
• What was ‘SAM’? Did it have any role in
• Is the management any different in
tachyarrhythmia with SAM?
What is SAM?
• Anterior movement of mitral valve (either
of the leaflets) during systole.
– Mostly involves anterior leaflet
• Maximal anterior motion in HCM patients
occurs before maximal posterior wall
contraction—approximately two-thirds of
the way through systole
Why does it happen?
• Venturi effect: increased flow velocity in
• Anterior and inward displacement of
papillary muscle with elongation of valve
leaflet → creates slack in leaflet.
• Flow drag: Pushing force of the flow
• Timing of papillary muscle contraction
Three following features are necessary for SAM
Mitral-septal contact and obstruction: anterior position of
• Angle of flow onto the mitral valve, such that flow gets
behind the mitral valve (angle of attack)
• Chordal slack
Time course through systole after
Once the mitral valve touches the septum a narrowed
Pressure difference across the orifice becomes the new
hydrodynamic force across the mitral leaflet
This pressure difference pushes the leaflet further into the
septum, narrowing the orifice further
amplifying feedback loop is established that cycles for
much of ejection (longer in systole that it cycles, the
higher the gradient)
• On examination:
– LV heave
– S1 normal; S2 paradoxical splitting; S4
– Systolic ejection murmur – left sternal border
– Increases with valsalva, vasodilators
– Decreases with squatting, vasopressors
Parasternal long axis view
• Parasternal long axis
• HCM shows significant
– the interventricular
– posterior left ventricular
wall (PWLV); the echofree space behind the
posterior wall is a
pericardial effusion (PE).
RV: right ventricle
LV: left ventricle
LA: left atrium.
Hemodynamics in HCM with fixed
left ventricular outflow obstruction
HCM with variable left ventricular
outflow tract obstruction
AO = Descending aorta;
LV = Left ventricle;
After the third QRS complex, the ventricle
has more time to fill. Since there is more
time to fill, the left ventricle will have more
volume at the end of diastole (increased
Due to the Frank–Starling law of the
heart, the contraction of the left ventricle
(and pressure generated by the left
ventricle) will be greater on the
subsequent beat (beat #4 in this picture).
Because of the dynamic nature of the
outflow obstruction in HCM, the
obstruction increases more than the
left ventricular pressure increase. This
causes a fall in the aortic pressure as
the left ventricular pressure rises (seen
as the yellow shaded area in the picture).
• SAM was reported in late 1960’s with
Hypertrophic Cardiomyopathy (HCM)
– Thought to be specific for this entity
– Associated with LVOT obstruction
• Later both the findings were proven wrong
– SAM is present in 30-60% of HCM
– HCM with SAM: only 25-50% have LVOT obstruction
– SAM can be present in absence of HCM
• Diastolic dysfunction
• Prolongation of systolic ejection
• Reduction in stroke volume
• Disrupts MV functioning → MR
• Microvascular dysfunction
• Intolerant to tachyarrythmias
• Negative ionotropes –
Decrease LV ejection acceleration
Decrease hydrodynamic force on mitral valve
Decreased feedback loop
• Start with β-blockers :
– prolonging diastole → prolongs filling time.
But doesn’t decrease LVOT gradient.
• Verapamil: causes vasodilation.
– use in combination with β-blockers.
– Reduces gradient and prolongs exercise time
• Side effects
– Anticholinergic side effects (BPH)
– Can accelerate AV conduction ( hence always used
with beta blockers)
– Prolongs QT interval (stop if QTc increases by > 25%)
• Avoid with amiodarone/ sotalol etc.
Things to avoid
• Use diuretics with extreme caution !!!
– Reduce preload → increase obstruction
• Hemodynamics can be compromised by
• Positive inotropes
• DDD pacing with short AV delay
– Reduced LVOT gradient by 50%
– Not much difference in exercise capacity
– Can use in elderly or who have contraindication to
– Can use more negatively ionotropic medications since
they are now protected against bradycardia.
Alcohol ablation of septum
– Small balloon catheter is placed into a proximal septal artery
– Contrast is injected into the target septal perforator
– After occlusion of a septal perforator by a small balloon to prevent back
leakage, 1 to 4 mL of absolute alcohol in injected into the distal
– Balloon is left inflated for 5 to 10 minutes
36% reduction in acceleration
– Death in 0% to 4%
– LAD dissection
– Leakage of alcohol back into the LAD with LAD occlusion and large
– Complete heart block in 9% to 38%
Classical myotomy-myectomy is the ‘gold standard’
therapy for patients with severely symptomatic hypertrophic
obstructive cardiomyopathy more than three quarters of all
long-term survivors are in functional class I or II (New York
Heart Association) and overall survival after 18 years (mean
follow up 8.1 years) was 68%, with a linearized mortality rate
of 1.9% per patient-year.
Patients with obstructive HCM and mild or no symptoms have only
slight excess mortality.
However, patients with markedly elevated resting LVOT gradients
are at a high risk of heart failure and death.
These findings may have important implications for therapy, including
the timing of septal reduction therapy
SAM & HTN
• Frequency: 1% to 30% of patients with
LVH from HTN
• Maximal SAM occurred at the end of
systole with the mitral valve still anteriorly
• “Venturi effect” may be more pronounced
in this subgroup
SAM & HTN
– Vasodilators may increase SAM & LVOT
– Negatively ionotropes have beneficial effects
SAM & Diabetes
• In poorly controlled diabetics (HbA1c > 13)
– SAM occurred in 65% of diabetics with
β-stimulation (10% in controls)
– Possibly related to greater LV mass in those
who exhibit SAM
– May have implications in septic patients on
pressors – especially Norepinephrine
SAM & ACS
Compensatory hyperkinesis in non-infarcted
Reduced systolic diameter of the outflow
Provides substrate for obstruction
SAM & ACS
• Causes new murmur & can be confused
with VSD or papillary muscle rupture
• Clinical Implications !!!
– Inotropes & Vasodilators will WORSEN the
shock & increase LVOT obstruction
– Control heart rate & decrease
hyperadrenergic & hypercontractile state –
use BB or Vasoconstrictors ( to increase
afterload) – like phenylephrine
• SAM present in 8-35%
• Especially Dobutamine stress test*
• Mostly caused by mid cavity obstruction
but may have SAM & LVOT obstruction
• Consensus is that the changes are due to
catecholamine effect rather than a
physiological response (like exercise)
* The Effect of Dobutamine Stress on Left Ventricular Outflow Tract Gradients in Hypertensive Patients.
ANGIOLOGY 2004 55: 295
patients with symptomatic nonobstructive HCM have latent LVOTO.
This study suggests that all patients
with symptomatic non-obstructive
HCM should have exercise stress
General anesthesia !!
Altered ventricular and papillary muscle geometry
+ increased ventricular contraction and outflow
increase drag forces on the mitral valve leaflets
SAM after mitral valve surgery
• Up to 5% of mitral valve repair
• Mechanism: anterior displacement of the mitral
coaptation point, shifting the mitral leaflets
towards the LVOT
• Increased risk if
– excess of redundant tissue in the posterior leaflet
– ratio of anterior leaflet length to posterior leaflet length
of less than 1.3
– Insertion of an annuloplasty ring
SAM after mitral valve surgery
• What to do in these cases?
– Discontinuation of inotropes.
– Give appropriate fluid therapy.
SAM: Take home points
• SAM of Mitral Valve can be present in conditions other
than HOCM with important clinical applications.
• Hypovolemia/ Increased adrenergic flow/ Vasodilators
can accentuate latent SAM.
• ECHO is useful in diagnosis.
• Management consists of