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CMR of Non-ischemic Dilated and Restrictive Cardiomyopathies

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Presentation on theme: "CMR of Non-ischemic Dilated and Restrictive Cardiomyopathies"— Presentation transcript:

1 CMR of Non-ischemic Dilated and Restrictive Cardiomyopathies
Frederick L. Ruberg, MD Director, Advanced Cardiac Imaging Program Section of Cardiology, Department of Medicine Department of Radiology Boston University School of Medicine Boston Medical Center March 2, 2009

2 Utility of CMR in LV systolic dysfunction
Diagnosis Ischemic vs. Non-ischemic Etiology Prognosis Functional recovery with treatment Morbidity and mortality

3 Case Presentation 58 year old woman with class II-III HF symptoms referred for echo

4 Case Presentation

5 Why obtain CMR next? Precise quantification of LV and RV function and volumes from cine images Permit detection of improvement or decrement with treatment Quantification of associated valvular regurgitation Visualization of fibrosis or infarction (DE/LGE) Pattern of DE important to differentiate etiology Afford predictors of recovery Afford predictors of CRT efficacy

6 LGE Imaging: Initially for scar
Kim RJ et al., Circulation 1999

7 Fibrosis Imaging by DE/LGE
Imaging min after gadolinium (0.1 to 0.2 mmol/kg) Retained contrast in regions of fibrosis or infarction No contrast in normal myocardium Marholdt EHJ 2005

8 Ischemic DE Pattern by CMR
Marholdt EHJ 2005

9 Differentiation of Ischemic vs. Non-ischemic CMP
90 patients with CHF and LV dysfunction obtained cardiac cath and CMR 70% without CAD by cath 59% no DE 28% mid-wall DE 13% sub-endocardial DE (mis-assigned) 30% with CAD and history of MI 100% with sub-endocardial DE McCrohon et al. Circ 2003

10 Ischemic vs. non-ischemic
McCrohon et al. Circ 2003

11 Case Example – Ischemic or Non-
35 year old male with severe LV dysfunction TSH > 120

12 Case Example – DE images

13 Mid-wall enhancement Not subendocardial, does not follow infarction pattern Most frequently septal Lower signal intensity vs. MI Etiology and significance is controversial

14 Mid-wall enhancement: Morbidity and Mortality
101 patients with dilated CMR underwent CMR and were followed for 685 days 35% had mid-wall enhancement Increased risk of death or hospitalization (OR 3.4) No difference in mortality Increased likelihood of SCD/VT (OR 5.2) Persisted after correcting for LVEF Assomoul et al. JACC 2006

15 Mid-wall enhancement: Morbidity and Mortality
Assomoul et al. JACC 2006

16 Histologic correlate of mid-wall
Assomoul et al. JACC 2006

17 Mid-wall enhancement: Morbidity and Mortality
A. Mortality or hospitalization for CV cause B. Adjusted for age, LV/RV EF, LV volumes, digoxin VT VT Adjusted for LVEF Assomoul et al. JACC 2006

18 DE confers increased risk
65 patients with non-ischemic dilated CMP, EF < 35%, underwent CMR at baseline, followed for 17 months 42% showed LGE at baseline Non-ischemic pattern 44% of those with LGE had adverse event vs. 8% without (HF, ICD discharge, death) Wu, JACC 2008

19 DE and risk in non-ischemic CMP
Wu, JACC 2008

20 Functional Recovery with Medical Treatment
45 patients with CHF treated with beta-blocker, CMR with DE at baseline and 6 month follow-up 62% ischemic (of those 100% with DE) 38% non-ischemic (of those only 2% with DE) Transmurality of DE predicted contractile improvement, change in EDV and ESV Bello et al. Circ 2003

21 Functional Recovery with Medical Treatment
Bello et al. Circ 2003

22 Prediction of CRT outcome by CMR
23 patients who qualified for CRT underwent CMR at baseline, follow-up at 3 months for wall motion, 6 min walk, QOL 50% history of MI 57% demonstrated response DE amount lower in responders <15% of LV mass – 85% sens., 90% spec. Septal transmurality of < 40% - 100% sens/spec. White et al. JACC 2006

23 Prediction of CRT outcome by CMR
White et al. JACC 2006

24 Conclusions for dilated CMR
Absence of any DE is good (non-ischemic) Predicts likelihood of recovery Better outcomes with CRT Lower likelihood of events

25 Case Example – cine CMR

26 Case Example – DE CMR

27 Case Example Symptomatic improvement with ARB, beta blocker
Referred for CRT

28 Case Example 58 year old woman with class II-III HF symptoms referred for echo

29 Case Example HF with preserved LV function, grade II-III diastolic dysfunction

30 Differential Diagnosis
Etiology in this case is more important Hypertensive remodeling Hypertrophic Cardiomyopathy Infiltrative Cardiomyopathy Amyloidosis Storage disease (Anderson Fabry) Heavy metal deposition (hemochromatosis)

31 Utility of CMR Not necessary to define LV volumes, although mass quantification useful DE CMR Etiology Prognosis

32 Does LVH from HTN have DE?
83 patients with LVH from AS (25%), HTN (31%), and HCM (44%) underwent CMR DE seen in all etiologies AS 62%, HTN 50%, HCM 72% Only distinctive pattern from HCM Generally associated with increased mass Rudolph, JACC 2009

33 CMR in LVH Rudolph, JACC 2009

34 LVH with CHF

35 CMR in Amyloidosis Abnormally long myocardial T1 after Gd
Normal ≈ 1100 ms, amyloid ≈ 1400 ms Rapid clearance of gadolinium from blood pool, abnormal distribution kinetics Render blood pool dark Challenging to obtain optimal myocardial nulling Global, sub-endocardial pattern described Maceira et al. Circ 2005, Krombach, JMRI 2007

36 CMR in Amyloidosis Maceira: Circulation 2005

37 CMR in Amyloidosis Normal protocol Modified amyloid protocol
0.1 to 0.2 mmol/kg wait mins Modified amyloid protocol 0.1 mmol/kg wait 5 mins Diffuse DE, poor myocardial nulling

38 Diffuse DE seen in Cardiac Amyloidosis
Van den Driesen et al. AJR 2006

39 Performance of CMR in Amyloid
Sensitivity 80%, specificity 94%, PPV 92%, NPV 85% Vogelsberg et al, JACC 2008

40 CMR predictors of events
Amount or presence of DE does not predict mortality Amount of DE relative to LV mass does correspond to heart failure symptoms Ruberg et al, AJC 2009

41 CMR in Cardiac Amyloidosis
Amyloidosis with cardiac involvement Amyloidosis without cardiac involvement Ruberg et al,AJC 2009

42 CMR in Cardiac Amyloidosis
Ruberg et al,AJC 2009

43 CMR in Cardiac Amyloidosis
Intramyocardial T1 gradient between epi- and endo-cardium predictive of survival DE/LGE was not Maceira et al, JCMR 2009

44 CMR in Anderson Fabry 32 Fabry patients treated with a-glactosidase, CMR obtained at baseline, followed for 3 years 63% had fibrosis by DE, 27% did not Absence of fibrosis associated with improved function, reduced mass, improved exercise capacity Weidemann et al., Circ 2009

45 CMR in Anderson Fabry Weidemann et al., Circ 2009

46 CMR in hemochromatosis
T2* weighted imaging T2* abnormally shortened in iron deposition Widely explored for thalassemia With chelation treatment (deferoxamine/deferiprone), T2* increases correlate to functional improvement in LVEF Tanner et al. Circ 2007

47 Case Example – DE Images

48 Case example Diagnosis: Amyloidosis
LGE present but can tell patient not predictive of poor outcomes Underwent stem cell transplant in 2005, doing well today, HF symptoms are controlled

49 Conclusions In dilated CMP, absence of DE portends:
Recovery of LV function with medical treatment Lower likelihood of death or hospitalization for HF Higher likelihood of response to CRT In dilated CMP, presence of DE Identification of ischemic etiology and provides information in respect to revascularization recovery Increased risk of adverse event and lower CRT response

50 Conclusions In CMP with LVH/wall thickening, CMR with DE imaging can:
Identify etiology of CMP Follow response to treatment Associate with clinical outcomes CMR with DE is useful as baseline exam in all forms of cardiomyopathy

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