Download Role of CMR in heart failure and cardiomyopathy Late gadolinium

Role of CMR in heart failure and
cardiomyopathy
Hajime Sakuma
Department of Radiology, Mie University
Late gadolinium enhancement (LGE)
• LGE MRI can demonstrate site of
necrosis fibrosis or deposition of
necrosis,
abnormal substrates in myocardium
LGE MRI in a patient with sarcoidosis
Mewton N, JACC 2011;57:891
Etiophysiopathlogy of myocardial fibrosis
• Replacement/scarring fibrosis
− Acute/chronic ischemia,
ischemia infarction
− Sarcoidosis, Myocarditis
− Miscellaneous inflammatory disease
• Infiltrative interstitial fibrosis
− Amyloidosis,
y
Fabry
y disease
• Reactive interstitial fibrosis
− Hypertension, Valvular disease
− Diabetes, Aging
From Mewton N、JACC 2011;57:891
60-year-old man, Heart failure
Cine MRI
LGE MRI
Subendocardial LGE – Ischemic cardiomyopathy
80-year-old woman, Heart failure
Cine MRI
LGE MRI
No LGE – Dilated cardiomyopathy (DCM)
35-year-old man, Heart failure
Cine MRI
LGE MRI
Midwall LGE – Dilated cardiomyopathy (DCM)
Differentiation of heart failure related to dilated
cardiomyopathy and coronary artery disease
LGE
DCM
Ischemic LV
Absent
63 (59%)
0
Endocardial
8 (13%)
27 (100%)
Midwall
18 (28%)
0
McCrohon J, et al. Circulation 2003;108;54
Role of CMR as a gatekeeper to invasive coronary angiography
in patients presenting with heart failure of unknown etiology
120 patients with heart failure of unknown origin underwent CMR and CA
DCM: true DCM 83 (69%)
DCM: bystander infarct 4 (3%)
DCM: bystander CAD 4 (3%)
CAD: true CAD 27 (23%)
CAD: MI with unobstructed
coronary arteries on CA 2 (2%)
LGE MRI
CA
Sensitivity
100%
93%
Specificity
96%
96%
Accuracy
97%
95%
Assomull R G et al. Circulation 2011;124:1351-1360
Prognosis in dilated cardiomyopathy
LGE(-)
LGE(+)
• Midwall fibrosis is a predictor of all-cause mortality and
cardiovascular hospitalization, which is independent of
ventricular remodeling. In addition, midwall fibrosis by
CMR predicts SCD/VT.
Assomull RG. J Am Coll Cardiol 2006;48:1977
Effect of myocardial fibrosis on response
to medical therapy in DCM patients
Leong DP. Eur Heart J 2012;33:640
Effect of scar transmurality on outcome after
cardiac resynchronization therapy (CRT)
Transmural LV free wall scars is a negative predictor of
clinical outcome after CRT
Chalil S. Europace 2007;9:1031
Hypertrophic cardiomyopathy
(HCM)
• Prevalence of 1:500 in general
population.
• Most common genetic cardiovascular
disease.
80-year-old man, HCM
Cine MRI
LGE MRI
Stress perfusion MRI
Mie University Hospital
MR Imaging Protocol
• Cine MRI
− Short-axis,
Sh t i long-axis,
l
i four-chamber,
f
h b
LVOT
• Stress-rest perfusion MRI (optional)
• Late gadolinium enhanced MRI
− Short-axis, long-axis, four-chamber
• Phase contrast cine MRI (optional)
− Pressure gradient of LVOT
− Significant obstruction: PG>30mmHg
17-year-old women, HCM
Cine MRI
LGE MRI
Short axis
Long axis
Mie University Hospital
17-year-old women, HCM
Phase contrast
LVOT cine MRI
Pressure gradient 40mmHg
Magnitude
Phase contrast cine MRI
Mie University Hospital
MR findings in HCM
1.
1
2.
3.
4.
LV hypertrophy
Myocardial fibrosis
Diastolic dysfunction
Abnormal myocardial
y
p
perfusion
Late gadolinium enhancement in HCM
• 60-80% of HCM patients
• F
Frequently
tl observed
b
d in
i
hypertrophied regions,
especially junctions of
septum and RV free wall
• Patchy, mid wall location
• Increased myocardial
collagen
- Moon JC. J Am Coll Cardiol 2004;
43:2260
Relationship between amount of LGE on CMR
and segmental wall thickness
Late enhancement
(% segment)
30
20
10
0
<10
10-14
15-19
20-24
25-30
>30
Segmental wall thickness (mm)
LGE correlates positively with regional hypertrophy.
Choudhury L et al. J Am Coll Cardiol 2002:40;2156
Diastolic dysfunction in HCM
• Abnormal relaxation and
increased stiffness of LV
wall
ll
• Enlargement of left atrium
• Atrial fibrillation
− Heart failure
− Thromboembolism
Th
b
b li
71-year old man
Diastolic dysfunction measured by cine MRI in
relation to severity of fibrosis on LGE MRI
Time volume curve of LV
measured by cine MRI
30
140
100
80
60
40
20
0
0
200
400
600
800
1000
Time (ms)
dV/dt
600
20
10
0
400
200
dV/dt (ml/ss)
Severity index of LGE
E
LV volume
e (ml)
120
0
-200
y=-7.9x+31.0
y=-7.9x+31.0
r=-0.86,
r=-0.86, p<0.001
p<0.001
n=17
n=17
0
1
2
3
4
5
Peak filling rate measured by cine MRI
(EDV/s)
-400
-600
-800
0
200
400
600
Time (msec)
800
1000
Diastolic dysfunction was
inversely correlated with
severity of LGE.
Motoyasu M, Sakuma H et al. Circ J 2008;72:378-383
Mie University Hospital
Microvascular disease in HCM
• Pathology
− Abnormal intramural coronary artery with
thickened walls and narrowed lumens
• Perfusion study
− Rest myocardial blood flow (MBF) is
preserved.
preserved
− MBF during vasodilator stress is severely
impaired in HCM patients.
80-year-old man, HCM
Cine MRI
Adenosine stress perfusion MRI
4 chamber
Short axis
LGE MRI
4 chamber
Short axis
Mie University Hospital
MR measurement of myocardial blood flow
Phase contrast cine MRI of
coronary sinus
Cine MRI encompassing LV
1.2
LV mass (g)
1
0.8
0
8
0.6
Volume flow
(ml/sec)
0.4
Coronary
sinus flow
(ml/min)
0.2
0
Mean MBF
(ml/min/g)
-0.2
0
100 200 300 400 500 600 700 800
Time after R wave m
( sec)
Kawada N, Sakuma H. Radiology. 1999;211:129-135
Myocardial blood flow (MBF) at rest and
during dipyridamole stress in HCM
Control subjects
*
300
HCM patients
3
3
2.5
2.5
2
2
1.5
1.5
1
1
0.5
0.5
*
* p<0.01
4
*
3
200
2
100
1
0
0
LV Mass (g)
0
Rest MBF (ml/min/g)
0
Stress MBF (ml/min/g)
Flow reserve
MBF during dipyridamole stress and myocardial flow reserve were
significantly reduced in patients with HCM.
Kawada N, Sakuma H, et al. Radiology. 1999;211:129-135
Mie University Hospital
Apical HCM
Cine MRI
Vertical long axis
4 chamber
Short axis
LGE MRI
Vertical long axis
4 chamber
Short axis
Mie University Hospital
Apical aneurysm in HCM
• Prevalence of 2% in HCM
patients
• Late gadolinium enhancement
of aneurysm wall
• Associated with increased
event rate (10%/year)
(
y
)
− Maron MS, Circulation 2008;118:1541
Annual HCM mortality by sudden
death, heart failure and stroke
• Sudden death
Arrhythmia
• Heart failure
End-stage HCM
• Stroke
Atrial fibrillation
Annual HCM mortality (%)
A
5
4
Sudden death
Heart failure
Stroke
3
2
1
0
5-15 16-25 26-35 36-45 46-55 56-65 66-75
>75
Age at initial evaluation (years)
81-year-old women, HCM
Cine MRI
LGE MRI
Short axis
Horizontal long axis
Is LGE useful for predicting prognosis of HCM?
Mie University Hospital
Prognostic significance of LGE in HCM
Without LGE
Event free surviv
val
1
0.8
0.6
With LGE
0.4
hazard ratio of 3.4 for
cardiovascular death or events.
0.2
0
1
2
3
4
5
6
Time after CMR (years)
Event: Cardiovascular death, unplanned cardiovascular admission,
sustained VT or VF, or appropriate ICD discharge
O’Hanlon R, et al. J Am Coll Cardiol 2010;56:867
Kaplan-Meier survival curves with regard to cardiac
mortality in HCM patients with and without LGE
Cumulative survival
1
Without LGE
0.95
0.9
With LGE
0.85
0.8
0.75
P-log-rank = 0.013
0.7
1
2
3
4
5
Time after CMR (years)
No patient without LGE suffered from any cardiac
death, including sudden cardiac death.
From Bruder O, et al. J Am Coll Cardiol 2010;56:875
Kaplan-Meier event free survival curves in
HCM patients with and without LGE
1.0
Without LGE (N=21)
Event free survival
0.8
With LGE (N=61)
0.6
0.4
P=0.951
Event:
E
t Cardiovascular
C di
l d
death,
th unplanned
l
d
cardiovascular admission, sustained VT,
appropriate ICT discharge
0.2
0
0
0
1000
2000
3000
4000
Time after CMR (days)
Mie University Hospital
Cumulative survival after initial diagnostic
evaluation in HCM patients at 20 years or older
Cumulative survival ra
ate
1.0
Expected according to US mortality rate
08
0.8
0.6
HCM population
0.4
0.2
0
5
10
15
Time from diagnosis (year)
20
Survival curve for HCM patients was not significantly different
compared with the expected survival curve derived for the general
US population after adjustment for age, sex, and race.
Maron BJ, et al. JAMA. 1999;281:650
Annual morality rate of HCM
• 3% to 6% in tertiary centers
centers.
− Patients are often referred due to high risk or
substantial symptoms
• 1% to 2% in community based
hospitals
69-year-old woman, Sarcoidosis
LGE MRI
LGE MRI in cardiac sarcoidosis
• High sensitivity
- Spensitivity100%,
S
iti it 100% Specificity
S
ifi it 78%1
• Distribution pattern is non-specific
- Subendocardial, transmural, midwall,
subepicardial, or multiple patchy 2
• LGE d
does nott iindicate
di t activity
ti it off the
th
disease
1. Smedema J. Am Coll Cardiol 2005;45:1683
2. Patel MR. Circulation. 2009 ;120:1969
LGE in Sarcoidosis
・CAD-type
・Non-CAD-type
Patel MR. Circulation. 2009 ;120:1969
Etiophysiopathlogy of myocardial fibrosis
• Replacement/scarring fibrosis
− Miscellaneous inflammatory disease
− Acute/chronic ischemia, infarction
− Sarcoidosis, Myocarditis
• Infiltrative interstitial fibrosis
− Amyloidosis,
y
Fabry
y disease
• Reactive interstitial fibrosis
− Hypertension, Valvular disease
− Diabetes, Aging
Amyloidosis
LGE MRI
Mie University Hospital
Amyloidosis
• LV wall thickening
g
• Subendocardial LGE including
RV side of the septum
• Abnormal thickening and
enhancement in atrial wall and
atrial septum
y washout of blood signal
g
• Early
• Amyloid deposition in
interstitial space.
Maceria AM, Circulation 2005;111:186
Mie University Hospital
68-yeara-old woman, Fabry disease
LGE MRI
Black blood T2-weighted MRI
Courtesy by Kunihiko Teraoka, Tokyo Medical University
Fabry’s disease
• Alpha-galactosidase A deficiency, resulting
in glycosphingolipids deposition.
• X-linked but female heterozygotes may
develop disease
• LGE MRI distribution
− Basal infero-latedal segment
Cobelli FD,, AJR 2009;192:W97
;
Moom JC, Eur Heart J 2003:24:2151
• Differentiating Fabry from HCM is
important because enzyme
replacement therapy is effective
Etiophysiopathlogy of myocardial fibrosis
• Replacement/scarring fibrosis
− Miscellaneous inflammatory disease
− Acute/chronic ischemia, infarction
− Sarcoidosis, Myocarditis
• Infiltrative interstitial fibrosis
− Amyloidosis,
y
Fabry
y disease
• Reactive interstitial fibrosis
− Hypertension, Valvular disease
− Diabetes, Aging
50-year-old man
Aortic stenosis, Normal coronary artery
Cine MRI
Mie University Hospital
50-year-old man
Aortic stenosis, Normal coronary artery
LGE MRI
Mie University Hospital
Acknowledgments
Mie University Hospital
−
−
−
−
−
−
−
−
Kakuya Kitagawa, MD
Masaki Ishida, MD
Motonori Nagata, MD
Kan Takeda, MD
Shinichi Takase, RT
Hiroshi Nakajima, MD
Shiro Nakamori, MD
Masaaki Ito, MD
Matsusaka Central Hospital
− Yasutaka Ichikawa, MD
Tokyo Medical University
- Kunihiko Teraoka, MD