Chapter 8 - Cardiomyopathies and Myocarditis

Congratulations! You have passed with a score of 81 % - 21 out of 26 .

1. Case 1: Young patient presenting with syncope and breathlessness - ?HCM. Look at the
pilots.
There is dextrocardia.
The pulmonary artery is dilated.
There is coarctation of the aorta.
There is evidence of thoracic sarcoid.
The pulmonary artery arises of the left ventricle (transposition).
Correct. The pulmonary artery is dilated. There is no coarctation and normal VA connections.
We can't see any MRI evidence of thoracic sarcoid.
2. Case 1: Young patient presenting with syncope and breathlessness - ?HCM.
This scan does not exclude SAM and LVOT obstruction.
CMR is superior to ECHO in measuring peak gradients in HCM (enevlope
shape/peak).
There is SAM and LVOT at rest.
The distribution of the hypertrophy is symmetrical.
There is no cavity obliteration.
Correct. There is marked LVH. The distribution of the hypertrophy is asymmetric septal
hypertrophy (ASH). The papillary muscles are hypertrophied. This scan does not exclude SAM
and LVOT obstruction - it excludes them at rest, under today's loading conditions. There is
extensive cavity obliteration - the last 5cm at least of mid to apical LV cavity. This could be
associated with mid-cavity obstruction. Echo is often better for measuring peak gradients in
HCM as the obstruction is often dynamic without a laminar jet core, and Doppler detects the
envelope shape and peak better than CMR (but CMR can be better in determining the location
and relative importance of complex, multilevel obstruction).
3. Case 1: Young patient presenting with syncope and breathlessness - ?HCM. Note: There
are a number of research sequences here, eg rest perfusion, that we are going to ignore.
Look now at the LGE images, series 25 to 35.
% LGE determines whether an ICD will be implanted or not.
The LGE distribution in this patient is assosciated with coronary artery territory.
There is LGE involving at least 20% of the total myocardium.
There is LGE involving at least 50% of the myocardium.
This patient is unlikely to fufil criteria for an ICD.
Correct. TI scout - sometimes used to help select the right TI for LGE imaging (TI is an imaging
parameter. When set correctly, remote, non-infarcted, normal myocardium is nulled making it
black). There is extensive LGE - just look at that 2 chamber view. This is not in the distribution
found in coronary artery disease. Here is clearly more than 20% of total myocardium. Extensive
LGE in HCM is associated with progressive disease and heart failure in HCM, but also with the
onset of AF and malignant arrhythmias. Its significance should be taken in the context of overall
risk stratification. Here, we have already been told of 1 risk factor, syncope, so it is likely that
this patient will fulfil criteria for an ICD.
4. Case 1: Young patient presenting with syncope and breathlessness - ?HCM.
The septo-marginal trabeculation is hypertrophied.
There is no obvious RVOT.
There is basal RV cavity obliteration.
ICD insertion should pose no difficulty in this patient (should it be needed/indicated).
Incorrect. The septo-marginal trabeculation is hypertrophied. This is the large chunk of muscle
on the RV side of the basal antero-septum jutting into the RVOT. The anatomy exists for RVOT
obstruction - perhaps that's why the pulmonary artery is dilated. There is apical RV cavity
obliteration - this could cause difficulty positioning an ICD lead, if it should be needed/indicated.
5. Case 2: Body builder with history of steroid and growth hormone use, presenting with
decreased exercise tolerance and breathlessness. Concerning the extra-cardiac anatomy:
There is a degree of focal coarctation of the aorta.
There is prominent muscular development of the pectoral girdle.
The aorta is congenitally small in this patient.
This patient has evidence of chronic kidney disease.
This patient has pectus carinatum.
Correct. There is prominent muscular development of the pectoral girdle - this could imply
athleticsm, and athletic heart or, on occasions, may suggest an individual who uses performance
enhancing drugs. There are small (<1cm) bilateral pleural effusions. There is a pericardial
effusion - the consequences of which we will explore in a later question. The aorta does look
small compared to the heart - but this is almost always because the heart is enlarged rather than
the aorta small. But since you mention it, the HASTE stack and white blood pilots show there is
no coarctation - and no collateralisation. The HASTE stack is of poor quality - it may be that the
surface coils were not switched on - the rest of the scan is okay. The kidneys are not small.
6. Case 2: Body builder with history of steroid and growth hormone use, presenting with
decreased exercise tolerance and breathlessness.
The LV mass is going to be elevated by eccentric hypertrophy (elevated mass but with
normal wall thickness or modest increased thickness around a dilated cavity).
The LV mass is going to be elevated by concentric hypertrophy.
The heart looks minimally dyscoordinate.
The operator has accidentally repeated series 6.
Fold-over artefact is rectified by decreasing the field of view.
Correct. Series 6 was repeated in 7 and then 8 to remove the wrap or fold-over artefact by
increasing the field of view. The LV is very dilated and severely impaired. Looking at the short
axis stack, the heart looks severely dyscoordinate, even despite the freedom of movement given
to the heart by the pericardial effusion. The LV mass is going to be elevated by eccentric
hypertrophy (normal wall thickness or modest increased thickness around a dilated cavity).
7. Case 2: Body builder with history of steroid and growth hormone use, presenting with
decreased exercise tolerance and breathlessness.
There is at least moderate MR in this patient.
Calculating the LV stroke volume by formal analysis and subtracting the mitral
backward flow would give the regurgitant volume of the MR.
There is no mitral valve pathology.
In patients with severely impaired LV function, there is a tendency for MR (if present)
to look more severe than it actually is.
Calculating the LV stroke volume by formal analysis and subtracting the mitral
forward flow would give the regurgitant volume of the MR.
Correct. Series 7 shows that the left atrium does contract giving an atrial kick. There is mitral
regurgitation, best seen in series 8, but also 'end on' in the short axis stack eg series 12. This
looks significant - at least moderate. For patients with severely impaired LV function, there is a
tendency for the MR to look less severe than it actually is. Calculating the LV stroke volume by
formal analysis and subtracting the aortic forward flow would give the regurgitant volume of the
MR - but this has not been done here.
8. Case 2: Body builder with history of steroid and growth hormone use, presenting with
decreased exercise tolerance and breathlessness.
There is marked ventricular ventricular interaction.
There is significant septal flattening.
There is evidence of RA collapse in systole on the 4 chamber view.
There is evidence of RV collapse on the 4 chamber view.
Correct. The 4 chamber view, series 5, does show that the RA collapses in systole, an early sign
of tamponade. There is no RV collapse here. This is a trap for the unwary. On the 4 chamber
view, it looks like RV collapse, but when one looks at the short axis stack, this is from the
rotation of the heart in systole bringing a different part of the RV into the plane, creating a 2D
view mimicking collapse. Series 24 is a real-time short axis view during deep breathing. There is
no septal flattening and no ventricular ventricular interaction.
9. Case 2: Body builder with history of steroid and growth hormone use, presenting with
decreased exercise tolerance and breathlessness.
The likely diagnosis is DCM with severe dyscoordination and early decompensation.
The LGE images are of optimal quality.
The LGE images strongly indicate infarction and subsequent fibrosis as the primary
mechanism for disease in this patient.
 There is a small ventricular thrombus at the apex.
Correct. The LGE images are not perfect - there is some artefact (respiratory and probably CSF
ghosting). However, there is no infarction and no focal fibrosis. Overall this scan suggests DCM
with severe dyscoordination and early decompensation. There are no ventricular thrombi.
10. Case 3: This patient has a family history of cardiomyopathy, an abnormal ECG and a
known gene mutation which we will tell you about a the end. Look at the pilots and
localisers.
The localiser scans suggest decompensation from heart failure could be present.
There is small volume abdominal ascites.
There is para-tracheal lymphadenopathy.
The pilot scans do not support a case for sarcoid.
There are parenchymal lung changes evident on the localisers.
Correct. There is not evidence of thoracic sarcoid here - no lymphadenopathy, no parenchymal
lung changes. There are no signs of heart failure (no ascites, no effusions, no pulmonary
oedema). The heart is in the right place and the thorax is not distorted - both can alter the surface
ECG.
11. Case 3: This patient has a family history of cardiomyopathy, an abnormal ECG and a
known gene mutation which we will tell you about a the end. Look at the long axis cines.
The apex is thinned and almost aneurysmal.
Despite altered myocardium, there are no obvious regional wall motion abnormalities.
Series 25 represents operator error in having repeated the 4 chamber view.
There is relative preservation of RV function.
Correct. The RV and LV are both abnormal. The RV is dilated and impaired. There appear to be
regional wall motion abnormalities - but we will look again at the short axis stack and the
transverse stack. The LV has regional wall motion abnormalities also - the LV apex is thinned
and almost aneurismal, and there are 2 other abnormal areas on the antero-lateral wall seen in the
4 chamber view. A modified 4 chamber view has been repeated (series 25) to precisely delineate
these by re-piloting on the short axis stack.
12. Case 3: This patient has a family history of cardiomyopathy, an abnormal ECG and a
known gene mutation which we will tell you about a the end.
The thinned areas of LV all thicken abnormally.
There is a regional aneursym of the LV only.
This patient demonstartes normal thickening of the LV with preserved function.
In addition to other abnormalities, there is subtle LVH also.
In series 26, in the area of the lateral LV thinning there is clear impression of "lighter"
myocardium.
Incorrect. Both the LV and RV are very abnormal. There are regional aneruysms of LV and RV.
There is subtle LVH also. Some of the thinned areas of the LV appear to thicken normally - but
be careful, this may be through plane movement rather than actual thinning with preserved
function. On some views - eg series 26, in the area of lateral LV thinning, there is an impression
of darker myocardium - intrinsically abnormal myocardium.
13. Case 3: This patient has a family history of cardiomyopathy, an abnormal ECG and a
known gene mutation which we will tell you about a the end. Look at the T1 weighted
images which alternate with fat saturation T1 weighted images (series 34 to 52). Focus
particularly on series 43 and 44.
There is a white blood/black blood image sequence taken by mistake.
There is localised fat infiltartion in the interventricular septum only.
This imaging technique is particularly adept to imaging pericardial fat.
There is 40% fat in the RV free wall.
There is fat infiltrating the LV lateral wall from the endocardial surface.
Correct. These images demonstrate fat. By adding or removing a fat saturation pre-pulse, these
make fat signal alternately white and dark. This can be best seen in subcutaneous or pericardial
fat. Here, there is unequivocal fat in the interventricular septum, but also the RV trabeculae and
infiltrating the LV lateral wall from the epicardial surface.
14. Case 3: This patient has a family history of cardiomyopathy, an abnormal ECG and a
known gene mutation which we will tell you about a the end. Look at the late gadolinium
images, 56 onwards.
There is extensive epicardial and mid myocardial LGE - exclusively representing
fibrosis.
Despite extensive pathology, the overall cardiac function is not impaired.
Genetic studies are not warranted in this patient.
This scan has pathognomic significance for ARVC.
CMR fibro-fatty abnormalities fufil diagnostic criteria for ARVC.
Correct. Sometimes these fragile sequences need tweeking and multiple images to get them as
right as they can be.
15. Case 4: This is an 80 year old with increasing breathlessness. Look at the anatomy and
cines.
There is no pericardial fluid.
There is no pericardial fluid. Incorrect. There are no pleural effusions. There is a trace
of pericardial fluid. The LV does indeed look hypertrophied but this is more
concentric rather than asymmetric (see first case). This hypertrophied muscle does not
There is no impairment of thickening.
There is hypertrophy that is more concentric than asymmetric.
There is asymmetric hypertrophy.
Incorrect. There are no pleural effusions. There is a trace of pericardial fluid. The LV does
indeed look hypertrophied but this is more concentric rather than asymmetric (see first case).
This hypertrophied muscle does not thicken well.
16. Case 4: This is an 80 year old with increasing breathlessness.
Long axis function is preserved.
The mitral valve descent in systole is almost absent.
The myocardial pathology is likely secondary to hypertensive heart disease.
The hypertrophy is confined to the LV.
Mitral valve descent is greater for the septum than the free wall.
Correct. Long axis function is very impaired. The mitral valve descent in systole is almost absent
– it should be more like 15mm for the free wall and 10mm for the septum. There is RVH also –
that makes this much less likely to be hypertensive heart disease.
17. Case 4: This is an 80 year old with increasing breathlessness. Go straight to the late
gadolinium images – ignore 23 to 37, there from are a research protocol.
There is extensive circumferential transmural LGE in the LV and RV.
There is extensive circumferential subendocardial LGE in the LV and RV.
There is extensive breathing artefact.
This pattern of LGE is indicative of coronary artery disease.
There is a discrepancy between blood and mid myocardium nulling.
Correct. These images appear to be of poor quality. There is some breathing artefact, but, for
example, series 52 onwards are clean. The LGE is not strikingly bright. The blood and mid
myocardium are nulling together. Series 52 is the best example – there is extensive
circumferential subendocardial LGE in the LV and RV. This is unlike that found in other
cardiomyopathies or coronary heart disease and is a very typical appearance.
18. Case 4: This is an 80 year old with increasing breathlessness.
This patient might be expected to have high levels of cardiac copper deposition.
This patient might be expected to have a mutation in Troponin if genetically tested.
This patient might be expected to have significant myocardial iron loading.
This patient might be expected to have Plakophilin mutation if genetically tested.
This patient might be expected to have senile TTR type amyloid if biopsed.
Correct. The combination of biventricular concentric hypertrophy/wall thickening, markedly
impaired biventricular long axis function, blood and myocardial simultaneous nulling after
contrast and global subendocardial LGE is typical for cardiac amyloid. Often effusions are also
present. LV impairment occurs later in the disease. This patient had senile TTR type amyloid
confirmed by biopsy.
19. Case 5: Take a look at this case of a 16 year old male, just doing their exams. He had
been admitted to hospital 2 weeks earlier with chest pain, ST elevation, a troponin T of 2
 (significantly elevated) but normal coronary angiography. Note: Some images (19 to 26)
have loaded in the wrong order.
The fold-over direction (wrap) does not encroach on the image in series 7.
Series 7 has the left atrial appendage in view.
Series 8 is an optimal 2 chamber cine view.
Series 7 is an optimal 2 chamber cine view.
Series 7 is a 2 chamber view and is correctly avoiding any LVOT in the image.
Incorrect. Series 7 is trying to be a 2 chamber cine, but the LVOT is creeping in, as is the RVOT.
The slice needs rotating so it better goes through the anterior and inferior walls, generating series
8. Also, the fold-over direction (wrap) appears to be head foot and is encroaching on the images -
best seen if you expand the view rather than just look at the thumbnail. Series 8 does have the
left atrial appendage in view.
20. Case 5: Take a look at this case of a 16 year old male, just doing their exams. He had
been admitted to hospital 2 weeks earlier with chest pain, ST elevation, a troponin T of 2
(significantly elevated) but normal coronary angiography. Note: Some images (19 to 26)
have loaded in the wrong order. Looking at all the cines and short axis stack.
There is well defined, moderate wall motion abnormality.
Long axis function looks moderately impaired.
The apex looks somewhat hypokinetic.
Overall LV function looks normal.
There is thrombus overlying the apex (hypokinetic).
Correct. Overall LV function does look normal. Long axis function looks normal. There is a
subtle regional wall motion abnormality – the basal inferior and inferolateral wall look somewhat
hypokinetic.
21. Case 5: Take a look at this case of a 16 year old male, just doing their exams. He had
been admitted to hospital 2 weeks earlier with chest pain, ST elevation, a troponin T of 2
(significantly elevated) but normal coronary angiography. Note: Some images (19 to 26)
have loaded in the wrong order. Look at the late gadolinium enhancement scans.
This scan is pathognomic of tako tsubo.
A history of heavy cocaine use in this young person is likely.
The LGE is patchy in distribution and subendocardial.
There is mid-epicardial LGE - ? myocarditis.
Despite the normal angiogram this pattern of LGE reflects infarction.
Correct. There is left ventricular LGE. This is not subendocardial, but mid or even epicardial. It
is most prominent in the basal to mid infero-lateral wall and looks patchy. This is not the
appearance of infarction and not the appearances of tako tsubo (where no or almost no scar is
evident) but of myocarditis.
 22. Case 6: New heart failure with peripheral oedema. One episode of slurred speech
within last month – MRI Brain reported as normal, EEG reported as being slightly outside
normal range. Echocardiogram – moderate concentric LVH of 17mm. Mildly impaired LV
function. Restrictive mitral inflow pattern with moderately dilated atria and possible RVH.
Referred for CMR to further delineate.
There is a raised left hemidiaphragm.
There are sternal wires visible.
There is moderate ascites.
Series 6 and 7 are blurred (gating issue), and have been repeated in series 8 and 9.
There is diffuse parenchymal lung changes.
Correct. There is no ascites and no pleural effusions. There is a trace of pericardial effusion.
Series 6 and 7 are blurred (?gating issue), and have been repeated in series 8 and 9.
23. Case 6: New heart failure with peripheral oedema. One episode of slurred speech
within last month – MRI Brain reported as normal, EEG reported as being slightly outside
normal range. Echocardiogram – moderate concentric LVH of 17mm. Mildly impaired LV
function. Restrictive mitral inflow pattern with moderately dilated atria and possible RVH.
Referred for CMR to further delineate.
The LV cavity is moderately dilated.
Radial function is very reduced.
Biventricular long axis function is very reduced.
There is evidence of regional wall motion abnormality in all 3 coronary artery
territories.
The LV ejection fraction is preserved so heart function is normal.
Correct. The LV is myocardium is hypertrophied (or should we say ‘thickened’ – see later). The
LV cavity is normal in size. Biventricular long axis function is very reduced. Radial function
appears preserved, but thickening is poor. We measured the EF at 62% and the maximum wall
thickness at 25mm. There are no regional wall motion abnormalities. The RV myocardium
appears thickened. It is heavily trabeculated towards the apex.
24. Case 6: New heart failure with peripheral oedema. One episode of slurred speech
within last month – MRI Brain reported as normal, EEG reported as being slightly outside
normal range. Echocardiogram – moderate concentric LVH of 17mm. Mildly impaired LV
function. Restrictive mitral inflow pattern with moderately dilated atria and possible RVH.
Referred for CMR to further delineate. Look at the late gadolinium enhancement images eg
series 44 to 46
There is subendocardial LGE involving part of the right ventricle.
This shows a typical transmural LGE pattern, well localised to the coronary artery
territories.
The papillary muscles have been spared from the disease process.
 The underlying disease proccess is likely myocyte infarction.
Correct. There is extensive late gadolinium enhancement – this is in an unusual pattern – it
appears subendocardial, including the papillary muscles and in the RV. It is not well defined like
infarction. In these images, the ‘normal’ myocardium and blood pool are both black.
25. Case 6: New heart failure with peripheral oedema. One episode of slurred speech
within last month – MRI Brain reported as normal, EEG reported as being slightly outside
normal range. Echocardiogram – moderate concentric LVH of 17mm. Mildly impaired LV
function. Restrictive mitral inflow pattern with moderately dilated atria and possible RVH.
Referred for CMR to further delineate. Look at the interesting sequence, series 39. This is a
‘TI scout’ to help find the right parameters for doing late gadolinium imaging. Slow it
down if you need to.
The first images are black (taken just after the 180 inversion pulse).
The inversion time is related to T2.
With time different tissues go black in order, depending on their inversion time.
The inversion time is related to T2*.
Correct. What happens is that the first images are bright (taken just after the 180 inversion
pulse), then with time different tissues go black in order, depending on their inversion time
(related to T1), before becoming white again – (technically this is a magnetisation prepared
SSFP cine with a prior inversion pulse, with image reconstruction being a magnitude
reconstruction without phase information).
26. Case 6: New heart failure with peripheral oedema. One episode of slurred speech
within last month – MRI Brain reported as normal, EEG reported as being slightly outside
normal range. Echocardiogram – moderate concentric LVH of 17mm. Mildly impaired LV
function. Restrictive mitral inflow pattern with moderately dilated atria and possible RVH.
Referred for CMR to further delineate.
There is biventricular disease with thickening, disproportionate long axis impairment
and highly characteristic late gadolinium enhancement and contrast kinetics - strongly
suggestive of cardiac iron loading.
This scan is almost pathognomic for cardiac amyloidosis.
Concurrent ascites and/or pleural effusions would be rare in the context of a scan like
this.
The blood nulls first, suggesting there is minimal extra-cellular space in the
myocardium for contrast.
Incorrect. The myocardium nulls first, which means, (roughly) that it has more contrast in it than
the blood does). This is a fairly unusual finding, (unless you scanned almost immediately after a
high dose of contrast rapidly delivered). It suggests there is a huge extra-cellular space in the
myocardium for contrast.