View
108
Download
0
Category
Preview:
DESCRIPTION
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. - PowerPoint PPT Presentation
Citation preview
CMR of Non-ischemic Dilated and Restrictive Cardiomyopathies
Frederick L. Ruberg, MDDirector, Advanced Cardiac Imaging Program
Section of Cardiology, Department of Medicine
Department of Radiology
Boston University School of Medicine
Boston Medical Center
March 2, 2009
Utility of CMR in LV systolic dysfunction
Diagnosis Ischemic vs. Non-ischemic Etiology
Prognosis Functional recovery with treatment Morbidity and mortality
Case Presentation 58 year old woman with class II-III HF
symptoms referred for echo
Case Presentation
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
LGE Imaging: Initially for scar
Kim RJ et al., Circulation 1999
Fibrosis Imaging by DE/LGE Imaging 10-20 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
Ischemic DE Pattern by CMR
Marholdt EHJ 2005
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
Ischemic vs. non-ischemic
McCrohon et al. Circ 2003
Ischemic Non-ischemic
Case Example – Ischemic or Non- 35 year old male with severe LV
dysfunction TSH > 120
Case Example – DE images
Mid-wall enhancement Not subendocardial, does not follow
infarction pattern Most frequently septal Lower signal intensity vs. MI Etiology and significance is controversial
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
Mid-wall enhancement: Morbidity and Mortality
Assomoul et al. JACC 2006
Histologic correlate of mid-wall
Assomoul et al. JACC 2006
Mid-wall enhancement: Morbidity and Mortality
Assomoul et al. JACC 2006
A. Mortality or hospitalization for CV causeB. Adjusted for age, LV/RV EF, LV volumes, digoxin
A. VT B. VT Adjusted for LVEF
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
DE and risk in non-ischemic CMP
Wu, JACC 2008
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
Functional Recovery with Medical Treatment
Bello et al. Circ 2003
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
Prediction of CRT outcome by CMR
White et al. JACC 2006
Conclusions for dilated CMR Absence of any DE is good (non-ischemic)
Predicts likelihood of recovery Better outcomes with CRT Lower likelihood of events
Case Example – cine CMR
Case Example – DE CMR
Case Example Symptomatic improvement with ARB, beta
blocker Referred for CRT
Case Example
58 year old woman with class II-III HF symptoms referred for echo
Case Example
HF with preserved LV function, grade II-III diastolic dysfunction
Differential Diagnosis Etiology in this case is more important Hypertensive remodeling Hypertrophic Cardiomyopathy Infiltrative Cardiomyopathy
Amyloidosis Storage disease (Anderson Fabry) Heavy metal deposition (hemochromatosis)
Utility of CMR Not necessary to define LV volumes,
although mass quantification useful DE CMR
Etiology Prognosis
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
CMR in LVH
Rudolph, JACC 2009
LVH with CHF
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
CMR in Amyloidosis
Maceira: Circulation 2005
CMR in Amyloidosis Normal protocol
0.1 to 0.2 mmol/kg wait 15-20 mins
Modified amyloid protocol 0.1 mmol/kg wait 5 mins
Diffuse DE, poor myocardial nulling
Van den Driesen et al. AJR 2006
Diffuse DE seen in Cardiac Amyloidosis
Performance of CMR in Amyloid Sensitivity 80%, specificity 94%, PPV 92%, NPV 85%
Vogelsberg et al, JACC 2008
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
CMR in Cardiac Amyloidosis
Amyloidosis without cardiac involvementAmyloidosis with cardiac involvement
Ruberg et al,AJC 2009
CMR in Cardiac Amyloidosis
Ruberg et al,AJC 2009
CMR in Cardiac Amyloidosis Intramyocardial T1
gradient between epi- and endo-cardium predictive of survival
DE/LGE was not
Maceira et al, JCMR 2009
CMR in Anderson Fabry 32 Fabry patients treated with -
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
CMR in Anderson Fabry
Weidemann et al., Circ 2009
CMR in hemochromatosis T2* weighted imaging
T2* abnormally shortened in iron deposition Widely explored for thalassemia
Tanner et al. Circ 2007
With chelation treatment (deferoxamine/deferiprone), T2* increases correlate to functional improvement in LVEF
Case Example – DE Images
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
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
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
Recommended