the “el” conductor...1 infiltrative cardiomyopathy: an essential role for cmr amit r. patel md,...
TRANSCRIPT
April 28, 2018
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Infiltrative Cardiomyopathy: An Essential Role for CMR
Amit R. Patel MD, FACCAssociate Professor of Medicine and RadiologyDirector Cardiac MRI and CT
Disclosures: Research support from PhilipsResearch grant & Speaker’s Bureau AstellasResearch grant from General ElectricResearch grant from Myocardial SolutionsOff-label use of contrast agentsOff-label use of adenosine/ regadenoson
The “El” Conductor
• 70 year old man with sarcoidosis presents with dyspnea on exertion, palpitations, and chest pain
• Past Medical History
– Diabetes
– Hypertension
– Hyperlipidemia
– Sarcoidosis (orbital and pulmonary)
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Electrocardiogram
The “El” Conductor: Echocardiography
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Screening Strategy for Cardiac Sarcoidosis: HRS Consensus Statement
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Birnie. HRJ 2014
La
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Detection Strategies and Outcomes for Cardiac Sarcoidosis
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Sensitivity Specificity
Symptoms 65% 57%
ECG 21% 81%
Holter 59% 58%
TTE 27% 98%
CMR 97% 100%
Kouranos. JACC Imaging 2017
• 321 patients with biopsy proven extra-cardiac sarcoid
• Screening with symptoms, ECG, Holter, TTE, and CMR with LGE
• Outcomes: all-cause death, sustained ventricular tachycardia, and hospitalization for CHF
• 30% of patients had LGE/ “cardiac sarcoid”
– Really myocardial damage of “some sort”
• Median follow up 7 years
– 7.2% had major event (hazard ratio 5.68)
• Presence of LGE is an independent predictor of events
– >25% event rate (4% per year)
• Echocardiography of limited prognostic value when added to symptoms and ECG
Infiltrative CardiomyopathyInfiltrative Cardiomyopathy
CMR for the Evaluation of Cardiac Sarcoidosis: A Meta-Analysis
6Coleman. JACC Imaging 2016
Predicting Composite Outcomes
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Imaging-Guided Immunosuppressive Therapy
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After 6 Weeks of Prednisone
Cardiac MRI FDG PET
Cardiac MRI FDG PET
Scar versus Inflammation: Which Predicts Outcomes Better?
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• 56 symptomatic patients with high suspicion for CS
– MRI+/ PET+ (20)
– MRI+/ PET- (16)
– MRI-/ PET- (20)
– MRI-/ PET+ (0)
• 2.6 year follow up (death and VT)
– 16 events, all but 1 occurred in LGE + group
Bravo. IJC 2017
LGE Status
PET Status
LGE and PET
Detection of Inflammation Using CMR: T1 and T2-mapping
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Puntmann. Radiology 2017
• 53 patients with extra-cardiac sarcoid and 36 volunteers
• CMR with LGE and T1- and T2-mapping
• Repeat imaging in subset of 40 patients
– 18 with anti-inflammatory tx
– 22 without anti-inflammatory tx
• Sarcoid patients had higher T1 and T2 than controls
• Patients who underwent treatment had significant reduction in T1 and T2
Crouser. J Invest Med 2016
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The “El” Conductor: Late Gadolinium Enhancement
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Base Apex
Diagnosis: Cardiac Amyloidosis (aTTR)Treatment Plan:- Diuretics- ?Doxycycline- ?Diflunisol- ?Tafamidis- ?Patisiran or ?Revusiran- ?Green Tea Extract
AmyloidosisAmyloidosis
Prevalence of Echo Abnormalities in Cardiac Amyloidosis
11Quarta. Circulation 2012
172 Patients with Cardiac Amyloidosis
The “El” Conductor
Prevalence of Cardiac Amyloidosis in Elderly Patients with AS
• 113 patients with significant aortic stenosis referred for CMR
– Median age 74 years
• 16% of patients had evidence of cardiac amyloidosis
– If only considering men, 32% had cardiac amyloidosis
• 7 of 9 patients with cardiac amyloidosis had low flow/ low gradient AS
• Mortality in patients with AS w/ CA was significant greater than those with AS w/o CA (56% vs 20%)
12Cavalcante. JCMR 2017
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Infiltrative CardiomyopathyInfiltrative Cardiomyopathy
Amyloidosis and Cardiac MRI: Late Gadolinium Enhancement
• Interstitial myocardial expansion by deposition of insoluble amyloid fibrils originating from misfolded protein
• Presence of circumferential LGE on CMR had sensitivity 80%, specificity 94%, PPV 92%, and NPV 85% when compared to endomyocardial biopsy
13Selvanayagam. JACC 2007Vogelsberg. JACC 2008
Infiltrative CardiomyopathyInfiltrative Cardiomyopathy
Risk Stratification Using LGE in Cardiac Amyloidosis
• 250 prospectively recruited patients
– 122 with aTTR Amyloid
– 119 with AL Amyloid
• Mean follow up 24 months; 27% died
• Transmural LGE predicted death with hazard ratio 5.4 [CI: 2.1-13.7]
• Findings independent of nt-proBNP, LVEF, E/e’, LV mass index
14Fontana. Circulation 2015
Monitoring Response to Therapy
15Martinez-Naharro. iJACC 2017
• 31 patients with AL cardiac amyloid
• Serial testing before and after chemotherapy
• Baseline: LGE present in 84% and ECV 54±11%
• Remission rates: Complete 36%, Very good partial 29%, Partial or none 39%
• Regression (decrease in ECV>2 std dev) occurred in 92% of patients with complete or very good partial remission
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Infiltrative Cardiomyopathy
• Admitted for congestive heart failure (LVEF 30%) and ventricular tachycardia.
• Past medical history significant for:
– Sickle cell disease requiring multiple blood transfusions
– Atrial fibrillation
– Chronic DVT
– Diabetes Mellitus
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37 year old with Sickle Cell Thalessemia
Infiltrative Cardiomyopathy
TE 2.6ms TE 4.9ms TE 7.2ms
TE 9.5ms TE 11.8ms TE 14.1ms
T2* Myocardium = 12ms
T2* Imaging
Infiltrative CardiomyopathyInfiltrative Cardiomyopathy
Myocardial Iron Overload and Ventricular Tachycardia:
Independent of Systolic and Diastolic Function
Wood. Blood 2004
• Moderate relationship between T2* and LVEF (r=0.52)
• No relationship between E/A, E’, E/E’, or Tei index
17Leonardi. JACC Imaging 2008
Infiltrative CardiomyopathyInfiltrative Cardiomyopathy
T2* and Cardiovascular Outcomes
18Kirk. Circulation 2009
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Infiltrative CardiomyopathyInfiltrative Cardiomyopathy
T2*-Guided Therapy Improves Outcomes in Thalessemia
19Modell. JCMR 2008
Introduction of Cardiac T2*
The Accountant
• 62 year old man with easy fatiguability and palpitations
• Review of systems:
– Hypohydrosis
• Past Medical History:
– Coronary artery disease
– Atrial fibrillation (paroxysmal)
• Family history
– Paternal grandfather, father, paternal uncles x2 all died suddenly at young age
• Exam with normal blood pressure, no JVD, no edema, normal heart sounds
• EKG with severe LVH with ST/T changes
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LVEF: 70%IVSt: 19mmE: 82 cm/secA: 45 cm/secDecel Time: 0.17 secE/A: 1.8e’: 5 cm/secE/e’: 16.4GLS -6.8%
The Accountant
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Diagnosis: Atypical Variant Fabry Disease
Native T1: 870msLGE 2-ChamberLGE 4-Chamber
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Native T1 Relaxation Times Are Reduced in Fabry Disease
22Pica JCMR 2014
Healthy Fabry w/o LVH
Fabry w/ LVH Mean T1= 853±50ms
Mean T1= 968±32ms
Mean T1= 904±46ms
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Putting it Together with Cardiac Magnetic Resonance
Patel. JACC Imaging 2017
Thank [email protected]