Role of CMR in heart failure and cardiomyopathy
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1 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 abnormal substrates in myocardium LGE MRI in a patient with sarcoidosis
2 Mewton N, JACC 211;57:891 Etiophysiopathlogy of myocardial fibrosis Replacement/scarring fibrosis Acute/chronic ischemia, infarction Sarcoidosis, Myocarditis Miscellaneous inflammatory disease Infiltrative interstitial fibrosis Amyloidosis, Fabry disease Reactive interstitial fibrosis Hypertension, Valvular disease Diabetes, Aging From Mewton N JACC 211;57:891
3 6-year-old man, Heart failure Cine MRI LGE MRI Subendocardial LGE Ischemic cardiomyopathy 8-year-old woman, Heart failure Cine MRI LGE MRI No LGE Dilated cardiomyopathy (DCM)
4 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%) Endocardial 8 (13%) 27 (1%) Midwall 18 (28%) McCrohon J, et al. Circulation 23;18;54
5 Role of CMR as a gatekeeper to invasive coronary angiography in patients presenting with heart failure of unknown etiology 12 patients with heart failure of unknown origin underwent CMR and CA DCM: true DCM 83 (69%) CAD: true CAD 27 (23%) DCM: bystander infarct 4 (3%) DCM: bystander CAD 4 (3%) CAD: MI with unobstructed coronary arteries on CA 2 (2%) LGE MRI CA Sensitivity 1% 93% Specificity 96% 96% Accuracy 97% 95% Assomull R G et al. Circulation 211;124: 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 26;48:1977
6 Effect of myocardial fibrosis on response to medical therapy in DCM patients Leong DP. Eur Heart J 212;33:64 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 27;9:131
7 Hypertrophic cardiomyopathy (HCM) Prevalence of 1:5 in general population. Most common genetic cardiovascular disease. 8-year-old man, HCM Cine MRI LGE MRI Stress perfusion MRI
8 Cine MRI MR Imaging Protocol Short-axis, long-axis, four-chamber, 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>3mmHg 17-year-old women, HCM Cine MRI LGE MRI Short axis Long axis
9 17-year-old women, HCM Phase contrast LVOT cine MRI Pressure gradient 4mmHg Magnitude Phase contrast cine MRI MR findings in HCM 1. LV hypertrophy 2. Myocardial fibrosis 3. Diastolic dysfunction 4. Abnormal myocardial perfusion
10 Late gadolinium enhancement in HCM 6-8% of HCM patients Frequently observed in hypertrophied regions, especially junctions of septum and RV free wall Patchy, mid wall location Increased myocardial collagen - Moon JC. J Am Coll Cardiol 24; 43:226 Relationship between amount of LGE on CMR and segmental wall thickness 3 Late enhancement (% segment) 2 1 < >3 Segmental wall thickness (mm) LGE correlates positively with regional hypertrophy. Choudhury L et al. J Am Coll Cardiol 22:4;2156
11 Diastolic dysfunction in HCM Abnormal relaxation and increased stiffness of LV wall Enlargement of left atrium Atrial fibrillation Heart failure Thromboembolism b b 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 dv/dt (ml/s s) LV volume (ml) Time (ms) dv/dt Time (msec) Severity index of LGE 2 1 y=-7.9x+31. r=-.86, p<.1 n= Peak filling rate measured by cine MRI (EDV/s) Diastolic dysfunction was inversely correlated with severity of LGE. Motoyasu M, Sakuma H et al. Circ J 28;72:
12 Microvascular disease in HCM Pathology Abnormal intramural coronary artery with thickened walls and narrowed lumens Perfusion study Rest myocardial blood flow (MBF) is preserved. MBF during vasodilator stress is severely impaired in HCM patients. Cine MRI 8-year-old man, HCM Adenosine stress perfusion MRI 4 chamber Short axis LGE MRI 4 chamber Short axis
13 MR measurement of myocardial blood flow Phase contrast cine MRI of coronary sinus Cine MRI encompassing LV LV mass (g) Volume flow (ml/sec) Coronary sinus flow (ml/min) Mean MBF (ml/min/g) Time after R wave (msec) Kawada N, Sakuma H. Radiology. 1999;211: Myocardial blood flow (MBF) at rest and during dipyridamole stress in HCM Control subjects HCM patients * p< * * * * * LV Mass (g) Rest MBF (ml/min/g) 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:
14 Apical HCM Cine MRI Vertical long axis 4 chamber Short axis LGE MRI Vertical long axis 4 chamber Short axis Apical aneurysm in HCM Prevalence of 2% in HCM patients Late gadolinium enhancement of aneurysm wall Associated with increased event rate (1%/year) Maron MS, Circulation 28;118:1541
15 Annual HCM mortality by sudden death, heart failure and stroke Sudden death Arrhythmia Heart failure End-stage HCM Stroke Annual HCM mortality (%) Sudden death Heart failure Stroke Atrial fibrillation >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?
16 Prognostic significance of LGE in HCM 1 Without LGE val Event free surviv With LGE hazard ratio of 3.4 for cardiovascular death or events 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 21;56:867 Kaplan-Meier survival curves with regard to cardiac mortality in HCM patients with and without LGE l Cumulative surviva P-log-rank =.13 With LGE Without LGE 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 21;56:875
17 Kaplan-Meier event free survival curves in HCM patients with and without LGE 1. Without LGE (N=21) Event free survival P=.951 With LGE (N=61) Event: Cardiovascular death, unplanned cardiovascular admission, sustained VT, appropriate ICT discharge Time after CMR (days) Cumulative survival after initial diagnostic evaluation in HCM patients at 2 years or older Cumulative survival ra ate Expected according to US mortality rate HCM population Time from diagnosis (year) 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;281:65
18 Annual morality rate of HCM 3% to 6% in tertiary 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
19 LGE MRI in cardiac sarcoidosis High sensitivity - Spensitivity1%, Specificity it 78% 1 Distribution pattern is non-specific - Subendocardial, transmural, midwall, subepicardial, or multiple patchy 2 LGE does not indicate activity it of the disease 1. Smedema J. Am Coll Cardiol 25;45: Patel MR. Circulation. 29 ;12:1969 LGE in Sarcoidosis CAD-type Non-CAD-type Patel MR. Circulation. 29 ;12:1969
20 Etiophysiopathlogy of myocardial fibrosis Replacement/scarring fibrosis Miscellaneous inflammatory disease Acute/chronic ischemia, infarction Sarcoidosis, Myocarditis Infiltrative interstitial fibrosis Amyloidosis, Fabry disease Reactive interstitial fibrosis Hypertension, Valvular disease Diabetes, Aging Amyloidosis LGE MRI
21 Amyloidosis LV wall thickening Subendocardial LGE including RV side of the septum Abnormal thickening and enhancement in atrial wall and atrial septum Early washout of blood signal Amyloid deposition in interstitial space. Maceria AM, Circulation 25;111: yeara-old woman, Fabry disease LGE MRI Black blood T2-weighted MRI Courtesy by Kunihiko Teraoka, Tokyo Medical University
22 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 29;192:W97 Moom JC, Eur Heart J 23:24:2151 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, Fabry disease Reactive interstitial fibrosis Hypertension, Valvular disease Diabetes, Aging
23 5-year-old man Aortic stenosis, Normal coronary artery Cine MRI 5-year-old man Aortic stenosis, Normal coronary artery LGE MRI
24 Acknowledgments 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
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