9/14/2019 Evaluation and Management of No Disclosures Heart Rhythm - PDF document

9/14/2019 Evaluation and Management of No Disclosures Heart Rhythm Disorders in Patients with Cardiac Sarcoidosis 2019 California Heart Rhythm Symposium Vasanth Vedantham MD, PhD Vasanth Vedantham MD, PhD Associate Professor Associate Professor Division of Cardiology Division of Cardiology Cardiac Electrophysiology Cardiac Electrophysiology Cardiac Sarcoidosis Often Presents Outline as Arrhythmogenic Cardiomyopathy 1. Arrhythmogenic Substrate in Cardiac Sarcoidosis 2. Management of Conduction Disease 3. Management of Ventricular Tachycardia 4. Prevention of Sudden Death 5. Management of Atrial Arrhythmias 6. Role of Resynchronization Therapy Heart Rhythm DOI: (10.1016/j.hrthm.2019.05.007) 1

9/14/2019 Regional Myocardial Involvement in Patterns of Regional Involvement in Sarcoidosis Contributes to Cardiac Sarcoidosis Arrhythmogenic Substrate Inflammation, Granuloma, and Scar Correlation of Imaging and Findings is an Arrhythmogenic Combination in the Electrophysiology Lab EARLY – Lymphocytic Infiltrate EARLY – Lymphocytic Infiltrate GRANULOMA PHASE GRANULOMA PHASE LATE - SCAR Muser et al JACC EP 2018 Favora et. al. Am J Cardiol 2009;104:571 – 577 2

9/14/2019 Local Signal Correlates with Outline Imaging Findings 1. Arrhythmogenic Substrate in Cardiac Sarcoidosis 2. Management of Conduction Disease 3. Management of Ventricular Tachycardia 4. Prevention of Sudden Death 5. Management of Atrial Arrhythmias 6. Role of Resynchronization Therapy Daniele Muser et al. JACEP 2018;4:291-303 Case 1 What is the Appropriate Next Step? A 32 year old previously healthy woman experienced episodic lightheadedness. An A. She is asymptomatic with normal LV function ambulatory monitor showed brief episodes of so just monitor closely for now. symptomatic 2:1 AV block. She underwent B. Initiate immunosuppression pacemaker implantation and over several months C. Upgrade her pacemaker to a defibrillator developed complete heart block with 100% D. Further risk stratification (imaging or EPS) to ventricular pacing. She was referred to UCSF, guide decision on whether to upgrade where chest CT showed hilar adenopathy and pacemaker to ICD cardiac MRI and PET were consistent with active cardiac sarcoidosis. The patient is now asymptomatic with normal LV function and no ventricular arrhythmia detected on her device or on an ambulatory monitor. 3

9/14/2019 Unexplained AV Block The Cause of AV Block Matters Complete Heart Block < 60 years of age 2 retrospective case series: Finnish Registry 11/32 with unexplained CHB < 60 had CS Age >18 and <55 18/72 with unexplained CHB has CS Outcomes: 25-35% of young and middle-aged patients with Cardiac Death unexplained CHB have cardiac sarcoidosis . Transplant VT/VF Nery et al. (2014) J Cardiovasc Electrophysiol, 25(8): 875-881 Kandolin et al. (2011) Circ Arrhythm Electrophysiol, 4(3):303-9 Kandolin et al. (2011) Circ Arrhythm Electrophysiol, 4(3):303-9 Before Treatment Case 4: 1 week into treatment Complete Heart Block, 100% V-pacing AV Conduction Present, prolonged PR, QRS 145, 30% V-pacing 4

9/14/2019 Case 4: 1 month into treatment Recovery of AV Block in CS PR normal, QRS 135, 1% ventricular pacing Workup for Unexplained AV Block Outline 1. Arrhythmogenic Substrate in Cardiac Sarcoidosis 2. Management of Conduction Disease 3. Management of Ventricular Tachycardia 4. Prevention of Sudden Death 5. Management of Atrial Arrhythmias 6. Role of Resynchronization Therapy 2014 HRS Expert Consensus Document on CS 5

9/14/2019 Case 2 Case 2: EKG A 45 year old physical therapist with no significant PMH experienced sudden-onset syncope while at work. EMS was activated, and he was found to be in a WCT that required cardioversion. ECG in sinus rhythm showed RBBB. TTE showed mild RV enlargement with normal biventricular function and coronary angiography was normal. Transferred to UCSF for EPS/ablation. Cardiac MRI showed RV and septal LGE. Chest CT with no adenopathy or pulmonary dz and no other evidence of systemic disease. What is the Most Appropriate Next Case 2: PET Scan Step? A. Proceed to VT ablation B. PET scan C. Endomyocardial biopsy D. Genetic testing E. Antiarrhythmics and ICD implant with close follow-up and ablation if breakthrough VT or ICD shocks 6

9/14/2019 Endomyocardial Biopsy Voltage-Guided Biopsy Low sensitivity because disease is patchy Try to biopsy area of abnormality Can do EPS and voltage map to guide Liang et al. (2014) JACC Heart Fail. 2(5):466-73 See also: Nery et al. (2013) Can J Cardiol. 29(8):1015 25 26 Voltage-Guided Biopsy When Should VT Prompt a Workup for Cardiac Sarcoidosis? Unexplained MMVT (not outflow tract) 2 case series: 4/14 unexplained MMVT had CS 18/103 unexplained VT had CS ~15-30% of patients with unexplained VT have cardiac sarcoidosis . Nery et al. (2014) Pacing Clin Electrophysiol, 37(3): 364-374 Tung et al (2015) Heart Rhythm, Aug 10, S1547-527 27 7

9/14/2019 VT in Active Cardiac Sarcoidosis VT in Active Cardiac Sarcoidosis monomorphic pleiomorphic ploymorphic Panda et al PACE 2015 VT in a Patient with No Active Approach to VT Cardiac Sarcoidosis Inflammation Established CS With VT FDG FDG PET Pos Neg VT No VT VT FDGNeg PET Escalate IMS 8

9/14/2019 Outcomes of VT ablation in CS Progression of Arrhythmogenic Substrate in CS After 3 years of Disease “Quiescence” by PET Pre-Treatment Kumar et al 2015 and Muser et al 2016. Circ AEP. Complex Confluent Biventricular Scar; Multiple VT Morphologies; 86% Recurrence Rate after 1 procedure; Overall effectiveness at 2 years ~50% inclusive of multiple procedures, 85% reduced VT Who Should Receive an ICD? Outline 1. Arrhythmogenic Substrate in Cardiac Considerations Sarcoidosis History of Sustained VT or VF 2. Management of Conduction Disease Left Ventricular Function 3. Management of Ventricular Tachycardia Presence of Delayed Enhancement on MRI 4. Prevention of Sudden Death Inducible Ventricular Tachycardia Need for Pacemaker 5. Management of Atrial Arrhythmias On Appropriate Medical Therapy 6. Role of Resynchronization Therapy On Immunosuppression ?Anyone with bona fide cardiac sarcoidosis 9

9/14/2019 Appropriate Therapies are Frequent Who Should Receive an ICD. in CS Patients with ICDs Penn Data: 38% Received Appropriate Therapies 15%/year Rate of Appropriate Therapies Colorado Data 33% Appropriate Therapies 12%/yr Rate in Primary Prevention Main Predictor was impaired LVEF. Many primary prevention patients received therapies. Who Should Receive an ICD. How do Guidelines Perform? University of Minnesota CMR Registry: 290 pts with sarcoidosis, clinical follow-up and cardiac MRI. 10

9/14/2019 Important Areas For Future Conclusions Research • Cardiac sarcoidosis is highly arrhythmogenic • We need RCTs! • Early diagnosis and treatment can reverse AV block in some cases and can affect device • Ablation strategies in CS selection • Assessment of disease activity and substrate • Patients with active inflammation and VT can be progression treated with immunosuppression • Understand the pathophysiology and immunology • Patients with VT but no active inflammation might in order develop targeted therapies benefit from catheter ablation. • ICDs are often indicated for primary or secondary prevention. Thank You 43 11