Hypertrophic CMO Flashcards
What does this image represent?
SAM (systolic anterior motion of the MV)
What is this image represent? Label
Label the image, What does the arrows mean?
What does the errors point to?
What does this image represent?
Brockenbrough- Braunward morrow
What does this image represent?
Dynamic flow obstruction on M mode
What does this image represent
Label
What does ash stand for?
Aymptoeida
Label
Label
Label
What is this?
ASH: asymmetric septahypertrophy
What does this image represent?
M-mode of HH-colours
What does this image represent?
HCM- Apical CM
What does this image represent?
HCM Mid ventricular
What does this image represent?
HCM Mid ventricular Spectral
What does this image represent?
HCM Mid ventricular Spectral
What is cardiomyopathy?
Primary disease of the myocardium, excluding myocardial dysfunction due to CAD, HTN, CHD, or valvular disease
All cardiomyopathies can be classified according to what? In what order?
- 1st physiology
- Etiology once known
What are characteristics of dilated CMO? 3
- Dilation - 4 chambers
- LV S+D dysfunction
- +/- RV dysfunciton
What are characteristics of hypertrophic CMO? 3
- ASH
- LVH
- Hyperdynamic and non-dilated LV
What are characteristics of restrictive CMO 3
- Decreased LV compliance (Restrictive filling DDFxn)
- CHF
- Systolic function may be preserved
What are characteristics of other CMOS besides dilated CMO, Hypertrophic CMO, and restrictive CMO? 3
- Arrhythmogenic RV dysplasia
- LV non-compaction
- Idiopathic
What is the abbreviation of Dilated CMO?
DCM
What is the abbreviation of hypertrophic CMO?
HCM
What is the abbreviation of Hypertrophic obstructive CMO?
HOCM
What is the abbreviation of restrictive CMO?
RCM
What is HCM?
Disease of the myocardium characterized by the presence of LVH in the absence of another cause
What is the etiology of HCM?
Inherited disease: X linked autosomal dominant - scan relatives
What is the most common genetic CMO?
HCM
What is the pathophysiology of HCM?
Increased muscle mass due to myofibril disarray
The mutated gene in HCM affects what? What does this lead to?
- The contractile elements of the sacromere
- Leads to inappropriate hypertrophy of the muscle
What are S/S of HCM? 5
- Possible asymptomatic
- Exercise intolerance
- Syncope/ fainting (HOCM)
- Angina
- Increased sudden risk of death
What does the ECG look like for HCM? 2
- Arrhythmias
- AV blocks and bundle branch blocks
In terms of HCM, what are some arrhythmias seen with ECG?
A fib- high prevalence
What is the AV node location for HCM?
IVS
What is often the first clinical manifestation of HCM?
Increased risk of sudden death
What does decreased exercise intolerance look like for HCM?
SOB and fatigue
HCM can be divided into two sub groups based on what?
Whether the hypertrophy is causing any LV outflow obstruction or not
In terms of HCM, Hypertrophy involving what is the most common and most likely to have an outflow obstruction?
Basal anterior septum
In terms of HCM if the hemodynamic effect of the LVOT PG is above 30 mmHg what does this mean? What if its not?
- Yes: Obstructive (HOCM)
- No: Non obstructive
Non-obstructive HCM has a potential for what?
LVOT obstruction to develop at rest over time
What needs to be done with every patient with HCM?
Valsalva
What are two types of HOCM?
- Dynamic
- Latent obstruction
What is a dynamic obstruction?
Obstruction occurs when MV leaflets come in contact with thick IVS
What is Latent obstruction?
Discoverable on effect or provoking maneuvers such as Valsalva
What are some HCM echo features in Parasternal? 2
- Wall thickness (IVS and PW)
- Potential for SAM
How big should the IVS be in a patient with HCM?
IVS > 15mm
What is some apical echo features in apical?
- Presence/ distribution of hypertrophy
- Assess for SAM (5C/3C)
What are some things we can do to get a better 2D assessment of HCM?
- Use off axis views if needed to see the full thickness walls clearly for measurement
- Colour plus 2D to assess where aliasing occurs
What is needed for a good assessment of HCM?
Good endocardial definition
1/3 of individuals that have HCM have what?
RV involvement
What do we do to map the highest velocity in the LVOT for HCM?
Use colour aliasing and PW, specifically increase colour scale to see highest velocity
With HCM, it is easy to confuse MR with what?
Increased LVOT velocity because the leaks last longer
Doppler with HCM may have a decrease SV with normal ejection fraction due to what?
Decrease in cavity size
What can we use doppler to do with HCM?2
- MR assessment
- Assess Diastolic dysfunction
What is a tip to measure peak velocity with HCM? (using CW)
Walk the pulsed wave sample volume from apex all the way to the AV Looking for the point where the flow accelerated. Then use CW to measure peak velocity
What can mimic a HCM heart?
Athlete heart
How does a athlete heart mimic HCM?
Heart remodeling in highly trained endurance athletes
Athletes hearts may still be normal despite having what? What are two examples?
- Increased parameters
- LV, RV, LA dimensions
- LV wall thickness
Athletes hearts look like what?2
- Maintains symmetric, normal shape, No SAM
- Normal or supranormal diastolic function
What are the parameters for normal LV wall thickness for M/F athletes?
- Male <16mm
- Female <12mm
What is HOCM?
Obstruction: Higher velocity in the LVOT seen with color and aliasing, mapped with PW and measured the peak velocity and PG with CW doppler
How do we determine persistent obstruction?
Obstruction at rest and valsalva
How do we determine provocable obstruction?
Mild obstruction at rest that gets worse with Valsalva
How do we determine Latent obstruction?
Near normal velocity at rest that increases with provocation
When would we see obstruction with latent obstruction?
Only with valsalva
What are three kinds of provocative maneouvers?
- Amyl nitrite
- Valsalva maneuver
- Stress test
How is amyl nitrite a provocative maneouver?
Lowers arterial resistances/ increases HR
How is valsalva a provocative maneouver?
Decreases ventricular filling
How is stress tests provocative maneouvers?
Increases HR
Why is stress tests not preferred for provocative maneouvers?
Risk of arrhythmias
HOCM depends on what?
Preload and afterload
What are HOCM obstructions increased by? 3
- Lower preload»_space; leading to LV cavity volume
- Increased contractility
- Afterload
Symptoms of HOCM worsen by what? (Drugs)
Diuretics (which reduce preload and make SAM and obstruction worse)
IHSS stands for what?
Idiopathic hypertrophic subaortic stenosis
What is IHSS?
A term previously used to describe the classic form of HOCM
How many ways are there to categorize SAM? What are they?
Three
1. Mild
2. Moderate
3. Severe
What is mild SAM?
Within 10mm of IVS
What is Moderate SAM?
Brief contact with IVS
What is severe SAM?
Prolonged contact with IVS
Degree of LVOT obstruction depends on what things? 3
- Closeness of the AMVL to the IVS
- Onset of SAM to the onset of AMVL contact with IVS
- Duration of AMVL/ IVS contact
In terms of Degree of LVOT with SAM, what question must we ask in relation to closeness of the AMVL to the IVS?
Does it actually touch the septum?
In terms of Degree of LVOT with SAM, what question must we ask about the onset of SAM to the onset of AMVL contact with IVS?
How long does it take from the time MV leaflets starts moving when it touches the IVS
In terms of Degree of LVOT with SAM, what question must we ask in relation to the duration of AMVL/IVS contact?
How long does the MV touch the IVS
In terms of LVOT obstruction and PVCs, following a ventricular ectopic beat, what happens? What is this called?
- There is a marked increase in the LVOT gradient
- Brockenbrough- bruanwald morrow sign.
Brokenbrough mraunwald morrow sign only occurs with what?
Dynamic obstruction
What is seen with HOCM on doppler?3
- High velocity LVOT flow
- Dynamic flow obstruction
- MR due to SAM
In terms of doppler and HOCM, what does dynamic flow obstruction look like?
Mid to late systolic peaking LVOT flow with gradient across LVOT
How do we map out dynamic flow obstruction for HOCM? 2
- Use PW to “map” level of obstruction
- Use CW to display peak velocity
In terms of HOCM doppler and dynamic flow obstruction, as the gradient S increases, what results?
Severity of the condition S also increases
In terms of HOCM, how is there MR due to SAM?
Lack of coaptation as the AML gets “sucked” toward the septum, the leaflet tips come apart
What does LVOT gradient look like on doppler?3
- Starts later
- Peaks mid-late
- “Dagger sign” of PW flow
How does MR look like on doppler compared to LVOT gradient? 5
- Starts earlier
- Ends later
- High velocity throughout
- Peaks mid
- Higher gradient/ velocity than LVOT
What is medical treatment options for HOCM?
Improve diastolic functions with Meds
What are drugs we should use to treat HOCM? 2
- Beta blockers
- Calcium blockers
What are types of drugs we should not use for HOCM treatment? why?
- Diuretics
- Reducing preload makes it worse
What are surgical options for HOCM treatment?3
- Septal myectomy
- Percutaneous intervention
- Implanted cardiac defibrillator (ICD)
What are some percutaneous interventions used for HOCM treatment?
Alcohol septal ablation
What does alcohol septal ablation do? 2
- Catheterization with alcohol injected into portion of LAD
- Kills and thins basal septal wall
What does implanted cardiac defibrillator do for HOCM treatment?
Prevention of sudden death
What is a myectomy?
A portion of the basal IVS is removed or ablated
How does a myectomy benefit HOCM?
Obstruction is eliminated
Myectomy may result in what? (In terms of HOCM)
May result in iatrogenic complications such as VSD, AI
Why is echo important in pre/ post op monitoring for HOCM?
- Pre-op: identify the problem
- Post-op: Assess the success of the procedure
What are three forms of HCM?
- ASH
- Mid ventricular hypertrophy
- Apical hypertrophy»LV systolic cavity obliteration
What does ASH stand for?
Asymmetric septal hypertrophy
What is ASH?
Thickened, echogenic IVS (>1.3cm), but normal PW
What is the IVS: PW ratio which determines abnormality?
> 1.3:1
What is the appearance of ASH?
- May have speckled appearance
- Narrowed LVOT
What is seen with ASH with HCM?
Decreased Systolic IVS thickening
What happens as a result of decreased systolic IVS thickening? (In terms of ASH with HCM)
Other walls hyperdynamic to compensate
What can be noted of the PLAX M-mode of HCM?2
- SAM
- Basal wall thickness ratio
What is the DDX from M-mode for HCM ASH?2
- Aging
- Sigmoid septum
What can be noted about the basal wall thickness ratio of HCM- ASH? What do we need to do?
- High risk >30mm = 40% risk sudden death
- Compare to SAX
Which part of HCM - Apical CM is involved?
Apical LV
What do we need to do to determine HCM- Apical CM? 3
- Image the true LV apex (contrast may enhance definition)
- Use colour doppler to define obstruction or increase velocities
- Look for apical infarction aneurysm, and clot
With HCM-Apical CM, 15% have what disorder?
Apical aneurysm
What kind of appearance does HCM- apical hypertrophy have in 2D?
Ace of spades appearance
What is HCM-Mid ventricular?
Obstruction in the mid-ventricular cavity
What happens in the LV with HCM-mid ventricular? 2
- LV contracts on itself
- Both sides of the LV walls touch each other
What does the LV cavity do during systole with HCM-Mid ventricular?
LV cavity obliterates
What is the DDX for HCM-mid ventricular
- Chronic HTN
- AS
What are echo finds of HCM- Mid ventricular?5
- Thick LV walls
- Increased velocities
- Late systolic peaking
- MR
- LVOT obstruction at higher HR
In terms of HCM- mid ventricular, the LV walls thicken where?
Pap level
Where is the increased velocities with HCM- mid ventricular?
Mid cavity with gradient
What is the late systolic peaking waveform look like in HCM?
Saw tooth waveform
In terms of HCM-mid ventricular, MR is concordant with what?
Degree of obstruction
