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

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Evaluation and Management of Heart Rhythm Disorders in Patients with Cardiac Sarcoidosis

2019 California Heart Rhythm Symposium

Vasanth Vedantham MD, PhD Associate Professor Division of Cardiology Cardiac Electrophysiology

No Disclosures

Vasanth Vedantham MD, PhD Associate Professor Division of Cardiology Cardiac Electrophysiology

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

Cardiac Sarcoidosis Often Presents as Arrhythmogenic Cardiomyopathy

Heart Rhythm DOI: (10.1016/j.hrthm.2019.05.007)

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Regional Myocardial Involvement in Sarcoidosis Contributes to Arrhythmogenic Substrate Patterns of Regional Involvement in Cardiac Sarcoidosis Inflammation, Granuloma, and Scar is an Arrhythmogenic Combination

Favora et. al. Am J Cardiol 2009;104:571–577 EARLY – Lymphocytic Infiltrate GRANULOMA PHASE LATE - SCAR

Correlation of Imaging and Findings in the Electrophysiology Lab

EARLY – Lymphocytic Infiltrate GRANULOMA PHASE

Muser et al JACC EP 2018

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Local Signal Correlates with Imaging Findings

Daniele Muser et al. JACEP 2018;4:291-303

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

Case 1

A 32 year old previously healthy woman experienced episodic lightheadedness. An ambulatory monitor showed brief episodes of symptomatic 2:1 AV block. She underwent pacemaker implantation and over several months developed complete heart block with 100% ventricular pacing. She was referred to UCSF, where chest CT showed hilar adenopathy and 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.

What is the Appropriate Next Step?

• A. She is asymptomatic with normal LV function

so just monitor closely for now.

• B. Initiate immunosuppression
• C. Upgrade her pacemaker to a defibrillator
• D. Further risk stratification (imaging or EPS) to

guide decision on whether to upgrade pacemaker to ICD

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Unexplained AV Block

Complete Heart Block < 60 years of age 2 retrospective case series: 11/32 with unexplained CHB < 60 had CS 18/72 with unexplained CHB has CS 25-35% of young and middle-aged patients with unexplained CHB have cardiac sarcoidosis.

Nery et al. (2014) J Cardiovasc Electrophysiol, 25(8): 875-881 Kandolin et al. (2011) Circ Arrhythm Electrophysiol, 4(3):303-9

The Cause of AV Block Matters

Kandolin et al. (2011) Circ Arrhythm Electrophysiol, 4(3):303-9

Finnish Registry Age >18 and <55 Outcomes: Cardiac Death Transplant VT/VF

Before Treatment

Complete Heart Block, 100% V-pacing

Case 4: 1 week into treatment

AV Conduction Present, prolonged PR, QRS 145, 30% V-pacing

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Case 4: 1 month into treatment

PR normal, QRS 135, 1% ventricular pacing

Recovery of AV Block in CS Workup for Unexplained AV Block

2014 HRS Expert Consensus Document on CS

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

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Case 2

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.

Case 2: EKG What is the Most Appropriate Next 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

Case 2: PET Scan

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Endomyocardial 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

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Voltage-Guided Biopsy

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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

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VT in Active Cardiac Sarcoidosis VT in Active Cardiac Sarcoidosis

Panda et al PACE 2015 monomorphic ploymorphic pleiomorphic

VT in a Patient with No Active Inflammation Approach to VT Cardiac Sarcoidosis

Established CS With VT FDG Pos FDG Neg PET No VT VT VT PET FDGNeg Escalate IMS

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Outcomes of VT ablation in CS

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

Progression of Arrhythmogenic Substrate in CS

After 3 years of Disease “Quiescence” by PET Pre-Treatment

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

Who Should Receive an ICD?

Considerations History of Sustained VT or VF Left Ventricular Function Presence of Delayed Enhancement on MRI Inducible Ventricular Tachycardia Need for Pacemaker On Appropriate Medical Therapy On Immunosuppression ?Anyone with bona fide cardiac sarcoidosis

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Appropriate Therapies are Frequent 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. Who Should Receive an ICD. How do Guidelines Perform?

University of Minnesota CMR Registry: 290 pts with sarcoidosis, clinical follow-up and cardiac MRI.

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Important Areas For Future Research

• We need RCTs!
• Ablation strategies in CS
• Assessment of disease activity and substrate

progression

• Understand the pathophysiology and immunology

in order develop targeted therapies

Conclusions

• Cardiac sarcoidosis is highly arrhythmogenic
• Early diagnosis and treatment can reverse AV

block in some cases and can affect device selection

• Patients with active inflammation and VT can be

treated with immunosuppression

• Patients with VT but no active inflammation might

benefit from catheter ablation.

• ICDs are often indicated for primary or secondary

prevention.

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Thank You