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PLAX
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2D/M-mode
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LV dimensions (LVIDs, LVIDd)
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LV cavity size may be normal or small
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(IVSd, LVPWd)
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May be normal
If > 12 mm concentric thickening in the absence of other
pathology (for example, hypertension, HCM or significant aortic
stenosis) may suggest infiltrative disease
Note: AL Amyloidosis particularly causes LV
increased wall thickness in the mild to moderate range whereas
TTR causes LV increased wall thickness in the
moderate to severe range (although there is overlap)
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LV mass
Relative wall thickness
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LV mass = 0.8 x {1.04 x [(LVIDd + LVPWd + IVSd)3 -
(LVIDd)3]} + 0.6g
Care should be taken to ensure accurate 2D measurements, as
errors are amplified by cubing when calculating LV mass
Relative wall thickness = (2 x LVPWd) ÷ LVIDd
In infiltrative cardiomyopathy there is concentric
hypertrophy
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*Granular or speckled appearance of myocardium
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Although this feature is known to be a characteristic feature of
cardiac amyloidosis, it is not a specific finding and hence should
not be used in isolation
Note: Low dynamic range, low grey scale compression and harmonic
imaging can mimic this appearance. Turning off 'harmonic' settings
may help to reduce over diagnosis
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* Aortic and mitral valve leaflet thickening
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Homogenous thickening of leaflets of all valves often seen
in amyloidosis
Note: Caution should be taken in this qualitative assessment
when using harmonic imaging, which may give rise to the appearance
of valve leaflet thickening (see note above
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* Pericardial and pleural effusions
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Frequently, trace or small pericardial and pleural effusions are
seen
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PSAX
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2D
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LV wall thickness at 4 points using clock face as reference (12,
3, 6, 9)
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2D frozen image at mid LV level at end diastole to demonstrate
concentric increased wall thickness
Note: Avoid inclusion of papillary muscles when measuring LV
wall thickness by 2D calliper
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Apical 4CH
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2D
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EF (Simpson's Biplane)
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Reduced in end stages, but may be normal or mildly reduced in
early disease
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* IAS thickening
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Visual assessment
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*Mitral and tricuspid valve leaflet thickening
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Visual assessment: homogenous thickening
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Apical 4CH and 2CH
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2D
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RA adn AL volumes and areas
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Measured at end ventricular systols and BSA indexed
Biatrial dilatation: RA area > 19cm2, LA volume >
28ml/m2
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Apical 4CH
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M mode
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MAPSE
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MAPSE <10mm
Reduced longitudinal function may be seen before deterioration
in global function assessed by EF
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Apical 4CH
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PW Doppler
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MV inflow pattern:
E/A ratio
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Severe diastolic dysfunction is more suggestive of an
underlying restrictive cardiomyopathy. Earlier in the natural
history of restrictive disease, abnormalities of LV filling by PW
Doppler of mitral inflow may be in the mild or moderate categories
of diastolic dysfunction. Please refer to the BSE diastolic
function assessment guidelines
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E deceleration time
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Short deceleration time
Note: normal diastolic filling sis extremely rare in cardiac
amyloidosis
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Apical 4CH
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PW TDI
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Mitral annulus:
e'
e'/a'
E/e' Sept and Lat
S'
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In restrictive filling:
Restrictive filling pattern with low e'
e'/a' <<1
E/e' (average of septal and lateral mitral annulus) > 13
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Earlier in the natural history of restrictive disease,
abnormalities of mitral annular PW TDI may be in the mild or
moderate categories of diastolic dysfunction. Please refer to the
BSE diastolic function assessment guidelines
Reduced systolic velocity
Reductions in TDI systolic and diastolic indices typically occur
earlier in the natural history of the amyloid disease process than
traditional echocardiographic measures, and may be a subclinical
marker when this condition is suspected
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Apical 4CH
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PW Doppler
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PV flow:
PVs/PVd
PVa
adur - Adur
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In restrictive filling:
PVs <<PVd
≥ 0.35 m/s
≥ 20 ms
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Apical 5CH
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PW or CW Doppler
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IVRT
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Short IVRT (<50ms) is in keeping with severe restrictive
filling, but in earlier stages of the disease process may be
prolonged or pseudonormal. Please refer to the BSE diastolic
function assessment guidelines
IVRT is quantified as the time interval between the end of LVOT
ejection and the onset of mitral inflow. This can be quantified by
PW or CW Doppler to record both mitral inflow and LVOT outflow
velocity profiles:
- PW Doppler: position the sample volume within the LVOT, but in
close proximity to the anterior mitral valve leaflet
- CW CW Doppler: position the Doppler beam in a hybrid
position that captures mitral inflow and LVOT outflow
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Subcostal
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2D M mode
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RV free wall thickness
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M-mode or 2D frozen image with zoom at end-diastole at the
level of the tricuspid valve chordae tendinae
≥ 5 mm RV free wall thickening is abnormal and is frequently
seen in cardiac amyloidosis
The administration of intravenous agitated saline may assist
in situations where endocardial definition is poor
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Apical 4CH and 2CH
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Deformation imaging
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Global and peak longitudinal systolic strain (optional but
extremely useful)
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Reduced with relative apical sparing, giving rise to a
characteristic 'bull's eye' appearance on speckle tracking
software*
Ensure high quality, optimized views for speckle tracking
post-processing. This should result in a frame rate that is
commensurate with optimal speckle tracking (at least > 80
fps)
Reductions in strain indices typically occur earlier in the
natural history of the amyloid disease process than traditional
echocardiographic measures, and may be a subclinical marker when
this condition is suspected
Due to inter-vendor variability, 'cut-off' values are not
currently advised, but must be interpreted relative to normative
data for individual speckle tracking packages
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