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MRI bei KardiomyopathienKardioUpdate 09.05.2019
Philip Haaf
Kardiologische Klinik, Universitätsspital Basel
MRI & Cardiomyopathies
1. Definition of Cardiomyopathy
2. Ischemic vs Non-ischemic cardiomyopathy
3. Tissue characterization with MRI for differential diagnosis of the
a.“dilated ventricle”
b.“thick ventricle”
4. Decision making: MRI for arrhythmogenic risk stratification
1. What is a cardiomyopathy?
Myocardial disorder in which the heart muscle is
structurally and/or functionally abnormal,
in the absence of
CAD
hypertension
valvular disease
congenital heart disease
sufficient to explain the observed myocardial abnormality.
McKenna WJ et al. Circ Res 2017
1. What is a cardiomyopathy?
Myocardial disorder in which the heart muscle is
structurally and/or functionally abnormal,
in the absence of
CAD
hypertension
valvular disease
congenital heart disease
sufficient to explain the observed myocardial abnormality.
McKenna WJ et al. Circ Res 2017
LGE
T1, T2, T2*,
ECV MappingFlow
Cine
Perfusion
Elliot P et al. Eur Heart J 2008 ESC Position Statement
What is clinically most important?
Morphologic Classification of Cardiomyopathies
Swoboda PP et al. Eur Cardiol 2017, Elliott P et al.
ESC WG on Myocardial and Pericardial Diseases.
Eur Heart J 2008
MOGES Classification
Morpho-Functional Phenotype
Organ/System Involvement
Genetic Inheritance Pattern
Etiology
Stage
Clinical practice (”99%”)
“Thick ventricle”
Hypertrophic cardiomyopathy (HCM)
Amyloidosis
Athlete’s heart
Anderson-Fabry
(Hypertensive heart disease)
“Dilated ventricle”
Dilated cardiomyopathy [NICM ICM]
Myocarditis (post-myocarditis)
ARVC
Iron overload CMP
Takotsubo-CMP
LV non-compaction
2. ICM NICM: LGE patterns
Non-coronary pattern of LGE Non-ischemic CMP
o subendocardium is typically spared
o often more patchy distribution
o localized in mid-wall and subepicardium
Coronary pattern of LGE Ischaemic CMP
o from subendocardium to epicardium
(ischaemic wave front) with increasing
coronary occlusion time
o matching with coronary distribution
mid-wall
patchy
endocardial
transmural
Ischemic vs. Non-ischemic
Images: University Hospital Basel
4Ch Cine 3Ch Cine
Pati
ent
APa
tien
t B
Images: University Hospital Basel
4Ch Cine 3Ch Cine4Ch LGE 3Ch LGE Conclusion
Ischemic
Non-ischemic
(DCM)
Pati
ent
APa
tien
t B
Ischemic vs. Non-ischemic
DCM – CMR features
• Systolic function↓
• Global LV (and RV), biatrial dilatation
• Mild eccentric hypertrophy or wall thinning
• exclusion of „ischaemic LGE“ / ischaemic heart disease
• Septal mid-wall LGE (25-30%); any LGE worse prognosis
In most cases lack of LGE consider T1 mapping
• No myocardial oedema
• Pleural effusion
Mid-wall
DCM
Consider (bystander) CAD in DCM if…
Marked regional wall motion abnormalities that
correspond with a coronary perfusion territory
Subendocardial or transmural LGE
Perfusion imaging can be difficult to interpret in DCM
thin myocardium
presence of scar
often slow blood flow
mri-blog.com
CMR – Histology
Wagner A et al, Lancet, 2003
CMR
Histology
Base Midventricular Apex
Dilated ventricle with LVEF↓↓ and no LGE excludes ICM (>90%).
LGE: breakthrough technique for CMR
Subendocardial Transmural“Ischaemic wave front”
Ischaemic Cardiomyopathy
Cardiac amyloidosis
Non-Ischaemic Cardiomyopathy
DCM
HCM
Myocarditis, Fabry
…ESC CMR pocket guide 2.0
LGE reference standard of myocardial scar and focal fibrosis.
Diffuse fibrosis may go undetected on LGE imaging.
Eitel I, et al. JACC 2014
LGE
Cardiac sarcoidSystemic Sclerosiss
ARVC
Normal myocardium
predominantly (≈75%)
cardiac myocytes
Interstitium (≈25%):
fibroblast, collagen,
endothelial cells, coronary
arteries
Normal ECV map
(≈ 25% ECV)
Normal LGE Myocardial infarction
increased collagen
synthesis with minimal
or no loss of viable
myocytes
Normal LGEHeterogeneous ECV map
green increased ECV >29%
Histopathology of myocardial fibrosis
Rathod R. et al. Circulation 2015
reversibleirreversible
3. Tissue characterization with MRI for DD of the “dilated ventricle”
Dilated cardiomyopathy [NICM ICM]
Myocarditis (post-myocarditis)
ARVC
Iron overload CMP
Takotsubo-CMP
LV non-compaction
Myocarditis /Non-ischemic myocardial inflammation
Typically inferolateral/inferior LGE
with a mid-wall to subepicardial
enhancement
Atypical cases of myocarditis with
transmural or diffuse LGE
Often concomitant LGE involvement
of the pericardium
Myocardial oedema only in the acute
setting
Images: University Hospital Basel
Fibrosis Oedema
Updated Lake Louise Criteria (“2 out of 2”)
Co
ntra
st-fre
eassessm
en
tfe
asib
le
(T1 a
nd
T2 m
appin
g)
3. Tissue characterization with MRI for DD of the “dilated ventricle”
Dilated cardiomyopathy [NICM ICM]
Myocarditis (post-myocarditis)
ARVC
Iron overload CMP
Takotsubo-CMP
LV non-compaction
LGE
RV 3 Ch “Inflow Outflow view”Triangle of dysplasia
(inflow, outflow, RV apex)
Right ventricle• RV dilatation• RVEF ↓• RV dyskinesia• Subtricuspid aneurysm
Left ventricle• Normal LV dimensions• Preserved LVEF• Normal regional wall
motion
SAX 4 Ch
Major Task Force CMR criteria
Regional RV akinesia or dyskinesia or dyssynchronous RV contractionand 1 of the following: RVEDVi ♂: ≥110 mL/m2 ♀: ≥100 mL/m2
or RV ejection fraction ≤40%
RV fibrofatty replacement (66% RV, 15% LV involvement)no longer part of task force CMR criteria but further
supporting the diagnosis
ARVC
Imaging alone not sufficient for definite diagnosis
Diagnosis based on multiple major and minor criteria:
histology, ECG, arrhythmias, family history
Not only a right-ventricular disease!
1. Classical ARVC
2. “biventricular” variant
3. Left-Dominant Arrhythmogenic CMP (LDAC) arrhythmias
with RBBB morphology (+ infero-lateral T wave inversion)
classical ARVC with LBBB morphology arrhythmia
DD: RV/LV volume ratio:
classical ARVC: ≥1.4; biventricular ca. 1:1; LDAC: <1
ARVC AC (Arrhythmogenic Cardiomyopathy)
Epsilon Wave
Biventricular/LV variant
Quadrangle of dysplasia
3. Tissue characterization with MRI for DD of the “dilated ventricle”
Dilated cardiomyopathy [NICM ICM]
Myocarditis (post-myocarditis)
ARVC
Iron overload CMP
Takotsubo-CMP
LV non-compaction
T2* mapping and iron overloadMortality of Thalassaemia major in the UK
T2* T2*
Heart and liver iron content
[ESC CMR pocket guide 2.0]
Blood
transfusion
Iron
chelation
Iron overload cardiomyopathy
Risk of developing heart failure • T2* <10 ms: high
• T2* 10-20 ms: intermediate
• T2* > 20 ms: low
Potentially reversible cause of heart failure under effective
therapy
Dilated phenotype majority of patients, impaired systolic
function
Restrictive phenotype non-dilated ventricles, preserved
systolic function, diastolic dysfunction, enlarged atria
T2* map
3. Tissue characterization with MRI for DD of the “dilated ventricle”
Dilated cardiomyopathy [NICM ICM]
Myocarditis (post-myocarditis)
ARVC
Iron overload CMP
Takotsubo CMP
LV non-compaction
The four different types of Takotsubo
Templin C et al. N Engl J Med. 2015
82%
15%
2%
1-2%
Basal
ballooning
„chestnut“
Apical
ballooning
Midventricular
ballooning
Transient systolic dysfunction with apical ballooning/akinesia and basal hyperkinesia.
CMR: typical Takotsubo pattern
4 Ch 3 Ch 2 Ch
No evidence of LGE or myocardial infarction.
Cin
eSSFP
T2 M
app
ing
LGE
Myocardial oedema in the areas of wall motion abnormality.
Tako-Tsubo!?
Systolic dysfunction with apical akinesia/dyskinesia and basal hyperkinesia.
Acute MI (type 2) mimicking Takotsubo.
4 Ch 3 Ch 2 Ch
Acute transmural MI with microvascular obstruction (prolonged vasospasm in apical LAD territory).
Cin
eSSFP
T2 M
app
ing
LGE
Myocardial oedema in the areas of wall motion abnormality.
MVO
3. Tissue characterization with MRI for DD of the “dilated ventricle”
Dilated cardiomyopathy [NICM ICM]
Myocarditis (post-myocarditis)
ARVC
Iron overload CMP
Takotsubo-CMP
LV non-compaction CMP
LV non-compaction CMP
Aetiology
a) incomplete compaction of spongy myocardium
LV Non-compaction cardiomyopathy (LVNC)
b) hypertrabeculation due to aberration of cardiogenesis
Excessive trabeculation cardiomyopathy (ET)
LV non-compaction CMP
Triad of Clinical Presentation
1. heart failure
2. thromboembolism
3. malignant arrhythmias/SCD
Normal pronounced trabeculation LVNC
LV non-compaction CMP
ESC CMR pocket guide 2.0
LV non-compaction CMP
ESC CMR pocket guide 2.0
In the context of intermediate pre-test probability!
Difficult diagnosis…
Patient with family history of
cardiomyopathy
• Marked trabeculation of all LV walls
except the interventricular septum
Most likely LV non-compaction CMP
Patient with no clinical features of
LVNC
• Marked LV trabeculation, primarily in
the lateral wall
• No other abnormalities
Most likely anatomical variant
A B
A B
ESC CMR pocket guide 2.0
Clinical algorithm
Nay Aung et al. JACC CV Imaging 2017
ICD?
OAC?
OMT?
LV non-compaction CMP
Conservative diagnosis
• (genetic) overlap with DCM, HCM, ARVC, IHD
Often over-diagnosed, particular in
• DCM (thin compacted myocardium)
• Afro-Americans
Excessive trabeculation + no other feature = benign
Current criteria may over-diagnose LV non-compaction.
New guidance is anticipated…
3. Tissue characterization with MRI for DD of the “thick ventricle”
c
Hypertrophic cardiomyopathy (HCM)
Amyloidosis
Anderson-Fabry
Athlete’s heart
(Hypertensive heart disease)
HCM
chordal SAM inferobasal crypts
hypokinesia ofhypertrophied segments
LGE of anteriorRV hinge point
Wall thickness (≥15 mm)?
SAM?
Crypts?
Hypokinesia of hypertrophy?
Fibrosis of hypertrophy?
Differential diagnoses
Hypertrophic CMP(1:500)
• 60%: asymmetric septal HCM
• 25-30%: symmetric concentric
HCM
• 10%: apical HCM (Yamaguchi)
apical obliteration of LV cavity
• midcavity HCM apical pouch
• 18%: RV hypertrophy
B
D
G
H
I
Why CMR in HCM?
2D Echo CMR
Non-diagnostic Apical HCM
18 mm 35 mm
SCD risk factor
Why CMR in HCM?
Precise assessment of wall thickness and extent/localisation of
hypertrophy
Detection and quantification of myocardial fibrosis ICD?
DD: Sarcomeric/familial HCM (sHCM) Phenocopies of HCM
HCM: #1 SCD in the young family screening
3. Tissue characterization with MRI for DD of the “thick ventricle”
Hypertrophic cardiomyopathy (HCM)
Amyloidosis
Anderson-Fabry
Athlete’s heart
(Hypertensive heart disease)
AL amyloidosis
Fabry disease
ATTR amyloidosis
sarcomeric HCM
EACVI CMR textbook 2018
Phenocopies of HCM
Mostly symmetrical and contiguous hypertrophy
Rare occurrence of LVOT obstruction
Typical pattern of LGE
Low native T1 value (Fabry fatty infiltration)
Phenocopies of HCM
Global
subendocardialPatchy
subendocardial
Amyloidosis Fabry
midwall
inferolateral
Tissue characterization
Normal native T1
map
„Fatty“ native T1
mapMidwall
Inferolateral LGE
Sado DM et al. Circ Cardiovasc Imaging 2013
Healthy Anderson-Fabry
Amyloidosis: Multi-modality imaging
Echo
- diastology
- AV stenosis
- strain
Tc-99m bone scan
- Perugini 2/3
ATTR
CMR
- ECV
- DD „thick
ventricle“
- (AL/ATTR)
(EMB)
- AL amyloidosis
- Subtypes, DD
Tissue characterization
Haaf P et al. JCMR 2016
ECV >>40% in
Amyloidosis
Normal ECV
nT1 in Fabry
ECV >30% in
HCM
3. Tissue characterization with MRI for DD of the “thick ventricle”
Hypertrophic cardiomyopathy (HCM)
Amyloidosis
Anderson-Fabry
Athlete’s heart
(Hypertensive heart disease)
Gray Zone (12-15 mm):Athlete’s Heart HCM
Athletes
True cellular hypertrophy
ECV↓
Hypertrophic CMP
Cellular disarray and extracellular matrix
expansion
ECV↑
Swoboda P. et al. JACC 2016
MRI & Cardiomyopathies
1. Definition of Cardiomyopathy
2. Ischemic vs Non-ischemic cardiomyopathy
3. Tissue characterization with MRI for differential diagnosis of the
a.“dilated ventricle”
b.“thick ventricle”
4. Decision making: MRI for arrhythmogenic risk stratification
4. Decision making: Arrhythmogenic risk
Would you rely on a decision making tool for ICD
implantation that misses 70-80% of future SCD?
70-80% of those who suffer SCD have an LVEF >35%!
Danish Trial: n=1116 NICM, EF<35%, no difference in
all-cause mortality (ICD vs. no ICD)
Furthermore, about 50% of all ICDs implanted are never
used (=potentially harmful: lead infection, inappropriate
shocks, atrial tachyarrhythmias…).
Adequacy of EF as (sole) gatekeeper to ICD?
Decision making:Arrhythmogenic risk
[Buxton AE, J Electrocardiol, 2016] [Stecker EC, JACC 2006] [Kober L, N Engl J Med, 2016]
Arrhythmogenic risk
[Disertori M, J Am Coll Cardiol Img, 2016] [Wu KC, Circ Cardiov Imag, 2017] [Klem I, JACC, 2012]
ICD discharge and SCD rates
Patients with preserved EF and
scarring on CMR have a similar
event rate to those with EF<30%
Patients with EF<30% but no scar
have a similar event rate to those
with preserved EF.
[Klem I, JACC, 2012]
Arrhythmogenic risk
Infarct/Scar Quantification
Meta-analysis (n=2.850, 3 y FU)
Infarct size >10% OR 5.6 [4.2-7.5]
[Disertori M, J Am Coll Cardiol Img, 2016] [Wu KC, Circ Cardiov Imag, 2017] [Klem I, JACC, 2012]
EF threshold to arbitrate ICD prescription:
lots of unused implants in pt’s with low EF
overlooked arrhythmic deaths in many patients with preserved EF
Outlook: Reliance on LVEF as the sole arbiter for ICD must end.
Better: LVEF + scar quantification for arrhythmogenic risk stratification.
Decision makingICD discharge and SCD rates
[Klem I, JACC, 2012]
Arrhythmogenic risk in HCM
HCM
Multi-parameter approach
Much better risk stratification
LGE & Arrhythmogenic/SCD Risk
Chan et al. Circulation 2014
7x
Arbitrator LGE in HCM
Maron BJ
JACC Cardiovasc Imag
2016
Summary: MRI & Cardiomyopathies
1. CMR: ideal imaging modality for tissue characterization and
cardiomyopathies
2. DCM: ICM vs NICM?
3. ARVC biventricular/LV forms AC
4. LVNC conservative diagnosis ET
5. HCM and its phenocopies
6. Amyloidosis multimodality imaging (Echo, CMR, bone scan)
7. Arrhythmogenic risk (ICD?)
a) DCM: LVEF LGE (No LGE vs. ≥10% LGE)
b) HCM: ESC risk calculator (No LGE vs. ≥15% LGE)
Differential diagnosis in LV hypertrophy (I)
Differential diagnosis in LV hypertrophy (II)
CMR
Arbitrator
Maron BJ
JACC Cardiovasc Imag
2016
Added value of CMR vs. 2D Echo
• Optimal visualization of
– LV apex (apical HCM, LV thrombus)
– Lateral wall (circumflex territory)
– Basal septum (early asymmetricseptal HCM)
– RV (ARVC, right heart infarction, GUCH, cardiac shunts)
