Outline 1. Basics of Cardiac Sarcoidosis 2. Diagnosis of Cardiac Sarcoidosis Evaluation and Management of 3. Management of Cardiac Sarcoidosis Patients with Suspected Cardiac 4. Ventricular Arrhythmia in Cardiac Sarccoidosis Sarcoidosis 2016 UCSF/Queen’s Hospital Innovative Procedures, Devices, and State of Vasanth Vedantham MD, PhD Assistant Professor the Art Care for Arrhythmias, Heart Failure, and Structural Heart Disease Division of Cardiology Cardiac Electrophysiology Systemic Sarcoidosis Patterns of Myocardial Involvement Granulomatous Disease of Unknown Etiology Prevalence ~ 10-20 per 100,000 Presents between 10 and 40 years of age Half noted incidentally on CXR Lung most commonly affected organ 1
Histology EARLY – Lymphocytic Infiltrate GRANULOMA PHASE Diagnosis LATE - SCAR Favora et. al. Am J Cardiol 2009;104:571–577 Diagnosis of Cardiac Sarcoidosis Case 1. 39 year old man developed mild chronic cough. A CXR showed hilar adenopathy. Biopsy of adenopathy showed noncaseating granuloma consistent with sarcoidosis. ECG showed RBBB with T wave inversions. A TTE was normal. An ETT showed excellent exercise tolerance. Should this patient receive further testing for cardiac sarcoidosis? 2014 HRS Expert Consensus Document on CS 2
Who should be evaluated? Cardiac Sarcoidosis Frequently Silent 5% of patients with sarcoidosis have clinically apparent cardiac involvement Variable numbers from autopsy studies 20-70% of patients with sarcoidosis have subclinical cardiac involvement. Cardiac involvement is the most common cause of death in patients with sarcoidosis. All patients with systemic sarcoidosis should be screened for CS 2014 HRS Expert Consensus Document on CS Screening for cardiac involvement ECG/Holter Infra-His Conduction System Disease Repolarization Abnormalities QRS Fragmentation Abnormal SAECG with Late Potentials Pseudoinfarction Frequent PVCs Findings can mimic ARVC. 2014 HRS Expert Consensus Document on CS 12 3
EKG TTE Can be normal Common Findings: WMA or Anneurysm Basal LV septal HK or thinning Global HK Valvular Regurgitation (with Pap Muscle Involvement) Right ventricular dilation/dysfunction pHTN (lung involvement or LHF) 14 MRI Imaging Utility in Cardiac Sarcoidosis ~25% of ECS have positive MRI findings LGE as a single test performs better than most other clinical criteria in detection of disease Patterns can mimic CAD or other myopathies Severity of MRI findings predict adverse events. PET Challenges RV Distinguishing scar from active disease ICDs and pacemaker dependent patients 15 4
Case 2. Case 2. In March 2011, she had an episode of syncope 42 year old woman well until November 2010, with no prodrome. She was taken to the ED, where when she developed episodes of sudden-onset she was diagnosed with complete heart block and anxiety accompanied by coughing and shortness a dual chamber pacemaker was implanted. PVCs of breath. A Holter was done and was interpreted and polymorphic NSVT were noted during the as normal. She was told to lose weight, then was implant. TTE showed normal function. Over the given antibiotics which did not improve the cough. next year, she had occasional palpitations and 2 She then complained of heart pounding and episodes of NSVT were recorded on her occasional palpitations, and was diagnosed with pacemaker. Coronary angiography was normal, anxiety and depression and started on SSRIs. and she was referred with the questions: Is this cardiac sarcoidosis? Who Should be Evaluated? Workup for Unexplained AV Block Complete Heart Block < 60 years of age 2 recent 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 2014 HRS Expert Consensus Document on CS 5
Nuclear Imaging F18-FDG-PET F-18 FDG PET Sensitivity comparable or superior to MRI Technique and adherence to pre-imaging diet are critical and can prevent over- or under-diagnosis Can be used to follow disease activity Can identify extra-cardiac sites amenable to bx 21 Case 3. Case 3: EKG A 45 year old man with no significant PMH presented with sudden-onset syncope while at work as a physical therapist. He regained consciousness but HR was 200 so taken to ED, where he was in a WCT and ultimately required cardioversion. Underlying ECG showed RBBB. TTE showed mild RV enlargement with normal biventricular function. Referred to UCSF for EPS/ablation. Cardiac MRI showed extensive LGE. Chest CT with no adenopathy or pulmonary dz. Could this be isolated cardiac sarcoidosis? 6
Case 3: PET Cardiac Sarcoid Who Should be Evaluated? 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 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 27 28 7
Voltage-Guided Biopsy Management 29 Immunosuppression. Immunosuppression: UCSF Approach No consensus on optimal approach 3 weeks of high dose prednisone, followed by taper over 10 weeks Standard of care is corticosteroids Methotrexate ?role for adjunctive steroid-sparing therapy -methotrexate Follow-up with PET scan in 6 months -TNF blockers TNF Blocker if persistent disease activity 8
Case 4. Immunosuppression – Why Treat? A 32 year old woman complained of lightheadedness to her PCP . She wore a Holter No Randomized Data! monitor which showed episodes of 2:1 heart block. She underwent pacemaker implantation and Observational Data: subsequently developed complete heart block with 1. Prevent Scarring/Heart Failure 100% ventricular pacing. She was referred to 2. Prevent Arrhythmias UCSF, where chest CT showed hilar adenopathy 3. Reverse AV Block and cardiac MRI and PET were consistent with 4. Prolong Life? active cardiac sarcoidosis. The patient was asymptomatic with normal LV function and no ventricular arrhythmia. Should this patient receive immunosuppression? Case 4: Before Treatment Case 4: 1 week into treatment Complete Heart Block, 100% V-pacing AV Conduction Present, prolonged PR, QRS 145, 30% V-pacing 9
Case 4: 1 month into treatment Recovery of AV Block in CS PR normal, QRS 135, 1% ventricular pacing Who Should Receive an ICD. History of Sustained VT or VF Impaired LV Function Risk Stratification and ICDs Imaging Findings (Scar Size) Inducible VT at EPS (controversial) Needs Pacing ?Anyone with bona fide cardiac sarcoidosis 10
Who Should Receive an ICD. Who Should Receive an ICD. 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. VT in Sarcoid Commonly Pleiomorphic or Multiple Morphologies Immunosuppression and Antiarrhythmics for Active Disease Catheter Ablation for VT in Quiescent CS or for refractory VT Management of VT 11
Outcomes of VT ablation in CS Example of VT in a Quiescent CS Pt 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 Natural History of CS: Active Research Questions in CS Cardiac Sarcoidosis Consortium 2014 HRS Expert Consensus Document on CS 12
Conclusions • Patients with systemic sarcoidosis, unexplained CHB or VT should be screened for CS • Although ECG and TTE can be suggestive or diagnostic, advanced imaging or guided endomyocardial biopsy is frequently required to make the diagnosis Thank You • No randomized data, but Immunosuppression for active disease can prevent deterioration in EF, improve ventricular arrhythmias, and reverse complete heart block • ICDs are usually indicated for primary or secondary prevention. Role of catheter ablation for VT remains to be fully defined, but is probably best used in quiescent disease 50 13
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