9/14/2019 Disclosures When to consider an ICD in the non-ischemic cardiomyopathy • Nothing to declare patient without arrhythmia? Sergio L. Pinski, MD, FHRS Cleveland Clinic Florida Weston, FL, USA Declining risk of sudden death in NICM SCD-HeFT and subsequent meta-analysis Desai et al. JAMA 2004;292:2874 Bardy et al. N Engl J Med 2005;352:2251 Kober et al. N Engl J Med 2017;377:41 1
9/14/2019 The DANISH study Khamis et al. J Clin Epidemiol 2019;113:147 Kober et al. N Engl J Med 2016;375:1221 Cochrane Meta-Analysis EHRA Survey shortly after DANISH El Moheb et al. Cochrane Database Systematic Reviews 2018 Haugaa et al. Europace 2017;19:660 2
9/14/2019 Older age limits the benefits of ICD Refining the indication-Risk stratification • Select patients with higher risk of sudden cardiac death but lower risk of death due to heart failure and competing comorbidities Elming et al. Circulation 2017;136:1772 Electrical parameters are mediocre in identifying NICM pts ar risk for sudden death Gulati et al. JAMA 2013;309:896 Goldberger et al. JACC 2014;63:1879 3
9/14/2019 Gulati et al. JAMA 2013;309:896 Halliday et al. J Am Coll Cardiol Img 2019;12:1645 Meta-analysis of LGE as predictor of ventricular Ongoing randomized trial arrhythmias or LV remodeling in DCM • Cardiac Magnetic Resonance GUIDEd Management of Mild- moderate Left Ventricular Systolic Dysfunction (CMR- GUIDE) • ~ 400 pts with ischemic or nonischemic cardiomyopathy, LVEF 36 to 50%, + LGE • Randomized to ICD vs ILR • End-point is composite of SCD or hemodynamically significant ventricular arrhythmia • 2023 Becker et al. JACC Cardiovasc Imag 2018;11:1274 4
9/14/2019 ”Non -ischemic ” cardiomyopathy • Acromegaly • Inflammatory/autoimmune • AL amyloidosis • PVC-induced • Alcoholic • LBBB-induced • Anthracycline • LV non-compaction • Arrhythmogenic LV • Muscular dystrophies • Chagas’ disease • Pacing-induced • “Burn out” HCM • Peripartum • Cocaine/metamphetamine • Post myocarditis • Genetic/familial dilated • Sarcoidosis • Hemochromatosis • Tachycardia-induced Correlation between mutated genes and Prognostic score for pts with lamin mutations cardiomyopathy phenotype Peters et al. Heart Lung Circ 2019;28:31 Wahbi et al. Circulation 2019;140:293 5
9/14/2019 Truncating filamin C (FLCN) mutations carry Reclassification of Risk of Sudden Death high risk of sudden cardiac death Cleland et al. JACC 2017:70:1028 Ortiz-Genga et al. JACC 2016:68:2440 My approach to ICD in nonischemic cardiomyopathy Patients with NICM and no fibrosis have excellent Detailed clinical and family history, deep phenotyping (CMR +), genotyping long-term prognosis with CRT-P alone (ie, MOGE(S) classification, JACC 2014;64:304) Familial DCM with SCD or mutation with high ICD risk of sudden death (LMNA, SCN5A, RBM20, FLCN) ICD Significant LGE in CRM? No ICD, treat Treatable condition? and reevalaute Low risk of competing mortality? ICD (younger age, no severe HF (or transplant listed), no comorbidities) Leyva et al. J Am Coll Cardiol 2012;60:1659 6
9/14/2019 CRT responders have lower risk of ventricular arrhythmia Interesting Clinical Dilemma • Younger patient with heart block and mild LV dysfunction requiring more or less urgent pacemaker (LMNA, SCN5A, sarcoidosis) • Family history? • Extracardiac features? Chest CT for sarcoidosis • Same day in hospital CMR generally not available • Turn around time for genetic test, biopsy too long • ? EP study. • DF-1 ICD lead for RV pacing with pacemaker- Not MRI compatible Saini et al. JACC Clin Electrophysiol2016;2:307 Conclusions • Current guidelines, based on LVEF, are not specific or sensitive to identify pts who benefit from ICD • “ Nonischemic ” cardiomyopathy should not be longer used. Appropriate disease characterization identifies pts with diffent risks, natural history and response to treatment • Absence of LGE in CMR identifies pts with much lower risk of sudden death, at any given LVEF • Older age decreases the benefit of ICDs • RCTs to expand indications are likely. However, large RCTs to restrict indications are not forthcoming. We will have to use clinical judgement. 7
9/14/2019 Genetic causes of dilated cardiomyopathy AF ablation and LV fibrosis: The CAMERA- MRI Study Prabhu et al. J Am Coll Cardiol 2017;70:1949 Kayvanpour et al. Clin Res Cardiol 2017:106:135 Prabhu et al. J Am Coll Cardiol EP 2018;4:999 Risk models in DANISH Proposed algorithm for high-risk mutations Kristensen et al. JACC Heart Fail 2019:(in press) Gacita & McNally. Circ Heart Fail 2019;12:e005850 8
9/14/2019 Meta-analysis of LGE as predictor of SD or ventricular arrhythmias in DCM Majority of “NICM” patients resuscitated from VF did not have fibrosis in CMR Di Marco et al. J Am Coll Cardiol HF 2017;5:28 Voscoboinik et al. PACE 2018;41:1109 CRT-P vs CRT-D in elderly pts with NICM Saba et al. Heart Rhythm 2019;16:1065 9
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