Disclosures: Research support from Philips Research grant & Speaker’s Bureau Astellas Research grant from General Electric Research grant from Myocardial Solutions Off-label use of contrast agents Off-label use of adenosine/ regadenoson Utility of Cardiac MRI in Heart Failure Amit R. Patel MD, FACC Associate Professor of Medicine and Radiology Director Cardiac MRI and CT Cardiac Magnetic Resonance: A Multi-Parametric Evaluation Other Myocardial Processes Cine CMR Myocardial Myocardial Ischemia Scar (LGE) Myocardial Myocardial Fibrosis Capillary Leak (T1-W) Edema (T2-W) (T1-mapping) CMR and Heart Failure | 2 The Mother Holter Monitor • 33 year old woman with no significant past medical history. • Following birth of 2nd child, she developed CHF and palpitations. EKG Predominant Rhythm = NSR Occasional PVCs (2% of total QRS complexes) Frequent Bigeminy (noted particularly with symptomatic palpitations) Rare PACs(<1% of QRS complexes) 1 SVT Run (8 beats) No pauses No evidence for AV dissociation or block CMR and Heart Failure | 3 The Mother: Echocardiogram Clinical Diagnosis: Peripartum Cardiomyopathy CMR and Heart Failure | 4 The Mother: Cardiac Magnetic Resonance Genetic Testing: PKP2 (plakophylin 2) and Asn557Asp, likely Pathogenic. CMR and Heart Failure | 5 Final Diagnosis: Arrhythmogenic Right Ventricular Cardiomyopathy Arrhythmogenic Right Ventricular Cardiomyopathy • Inherited cardiomyopathy characterized by fibro-fatty replacement of the RV myocardium • Increased risk of ventricular tachycardia and right heart failure • Typically due to autosomal dominant mutations in desmosomal genes with variable penetrance • Diagnosis - multifaceted approach – Family history/ genetics Br Heart J 1994;71:215-218 – Electrocardiogram/ rhythm abnormalities – Tissue characterization from RV myocardial biopsy – RV dysfunction & structural alterations from imaging • RV angiography • Echo • Cardiac MRI • CMR is the imaging modality of choice to evaluate ARVC because of its ability to assess RV morphology and function CMR and Heart Failure | 6 Misdiagnosis of ARVC using CMR Butterfly Apex More than 70% of patients referred to ARVC center were incorrectly diagnosed and did not meet criteria RV-Sternum Tethering Inferior Superior CMR and Heart Failure | 7 te Riele. JCMR 2014 Mimics of Arrhythmogenic Right Ventricular Cardiomyopathy Unmasked with CMR Partial Congenital Pectus Excavatum Secundum Type Atrial Septal Defect Absence of Pericardium Pectus Carinatum Breast Implants Pulmonary Hypertension CMR and Heart Failure | 8 Quarta. JCMR 2013 Mimics of Arrhythmogenic Right Ventricular Cardiomyopathy Unmasked with CMR Cardiac Sarcoidosis Myocarditis Anomalous Pulmonary Vein CMR and Heart Failure | 9 Quarta. JCMR 2013 Diagnosing Arrhythmogenic Right Ventricular Cardiomyopathy CMR Task Force Criteria ENTRY Criteria – Regional RV akinesia or dyskinesia or dyssynchronous RV contraction – AND one of following MAJOR Criteria – RVEDV/ BSA ≥110ml/m2 (male) or ≥100ml/m2 (female) – RVEF ≤40% MINOR Criteria – RVEDV/ BSA 100-110ml/m2 (male) or 90-100ml/m2 (female) – RVEF 40-45% CMR and Heart Failure | 10 te Riele. JCMR 2014 Quantifying RV Size and Function Using CMR CMR and Heart Failure | 11 Addetia: Circ Imag 2014 Inclusion of Tissue Characterization Using CMR May Increase Diagnostic Sensitivity for ARVC India Ink Sign LGE No Task Force Criteria Met LGE India Ink Sign LGE Minor Task Force Criteria Met T1-signal CMR and Heart Failure | 12 Aquaro. AJC 2016 Structural Stage of ARVC Predicts Arrhythmic Risk CMR and Heart Failure | 13 te Riele. JACC 2013 The “El” Conductor Electrocardiogram • 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) CMR and Heart Failure | 14 The “El” Conductor: Echocardiography CMR and Heart Failure | 15 Screening Strategy for Cardiac Sarcoidosis: HRS Consensus Statement Late Gadolinium Enhancement CMR and Heart Failure | 16 Birnie. HRJ 2014 Detection Strategies and Outcomes for Cardiac Sarcoidosis • 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) Sensitivity Specificity • Presence of LGE is an independent predictor of events – >25% event rate (4% per year) Symptoms 65% 57% ECG 21% 81% • Echocardiography of limited prognostic value when added to symptoms and ECG Holter 59% 58% TTE 27% 98% CMR 97% 100% CMR and Heart Failure | 17 Kouranos. JACC Imaging 2017 CMR for the Evaluation of Cardiac Sarcoidosis: A Meta-Analysis Predicting Composite Outcomes CMR and Heart Failure | 18 Coleman. JACC Imaging 2016 Imaging-Guided Immunosuppressive Therapy Cardiac MRI FDG PET After 6 Weeks of Prednisone Cardiac MRI FDG PET CMR and Heart Failure | 19 Detection of Inflammation Using CMR: T1 and T2-mapping • 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 Puntmann. Radiology 2017 Crouser. J Invest Med 2016 CMR and Heart Failure | 20 Cardiac MRI in Patients with ICD CMR and Heart Failure | 21 Singh. ACC 2017 The “El” Conductor: Late Gadolinium Enhancement Base Apex Diagnosis: Cardiac Amyloidosis (aTTR) Treatment Plan: - Diuretics - ?Doxycycline - ?Diflunisol - ?Tafamidis - ?Patisiran or ?Revusiran - ?Green Tea Extract CMR and Heart Failure | 22 Prevalence of Echo Abnormalities in Cardiac Amyloidosis 172 Patients with Cardiac Amyloidosis The “El” Conductor CMR and Heart Failure | 23 Quarta. Circulation 2012 Prevalence of Cardiac Amyloidosis in Elderly Patients with Aortic Stenosis • 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 aortic stenosis w/ cardiac amyloidosis was significantly greater than those with AS w/o cardiac amyloidosis (56% vs 20%) CMR and Heart Failure | 24 Cavalcante. JCMR 2017 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 CMR and Heart Failure | 25 Vogelsberg. JACC 2008 Selvanayagam. JACC 2007 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 CMR and Heart Failure | 26 Fontana. Circulation 2015 Monitoring Response to Therapy • 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 CMR and Heart Failure | 27 Martinez-Naharro. iJACC 2017 37 year old with Sickle Cell Thalessemia T2* Imaging TE 2.6ms TE 4.9ms TE 7.2ms • Admitted for congestive heart failure (LVEF 30%) and ventricular tachycardia. • Past medical history significant for: – Sickle cell disease requiring TE 9.5ms multiple blood transfusions TE 11.8ms TE 14.1ms – Atrial fibrillation – Chronic DVT – Diabetes Mellitus T2* Myocardium = 12ms CMR and Heart Failure | 28 Myocardial Iron Overload and Ventricular Tachycardia: Independent of Systolic and Diastolic Function • Moderate relationship between T2* and LVEF (r=0.52) • No relationship between E/A, E’, E/E’, or Tei index CMR and Heart Failure | 29 Wood. Blood 2004 Leonardi. JACC Imaging 2008 T2* and Cardiovascular Outcomes CMR and Heart Failure | 30 Kirk. Circulation 2009 T2*-Guided Therapy Improves Outcomes in Thalessemia Introduction of Cardiac T2* CMR and Heart Failure | 31 Modell. JCMR 2008 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 LVEF: 70% • Exam with normal blood pressure, no JVD, no edema, normal IVSt: 19mm heart sounds E: 82 cm/sec A: 45 cm/sec • EKG with severe LVH with ST/T changes Decel Time: 0.17 sec E/A: 1.8 e’: 5 cm/sec E/e’: 16.4 GLS -6.8% Clinical Diagnosis: Hypertrophic Cardiomyopathy CMR and Heart Failure | 32 The Accountant LGE 4-Chamber LGE 2-Chamber Native T1: 870ms Final Diagnosis: Atypical Variant Fabry’s Disease CMR and Heart Failure | 33 Native T1 Relaxation Times Are Reduced in Fabry Disease Healthy Fabry w/o LVH Fabry w/ LVH Mean T1= Mean T1= Mean T1= 904±46ms 853±50ms 968±32ms CMR and Heart Failure | 34 Pica JCMR 2014 54 year old woman with a few days of intermittent chest pain LAD Occlusion LAD Stent