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When to consider an ICD in the
non-ischemic cardiomyopathy
patient without arrhythmia?
Sergio L. Pinski, MD, FHRS
Cleveland Clinic Florida
Weston, FL, USA
SCD-HeFT and subsequent meta-analysis
Bardy et al. N Engl J Med 2005;352:2251
Desai et al. JAMA 2004;292:2874
Declining risk of sudden death in NICM
Kober et al. N Engl J Med 2017;377:41
The DANISH study
Kober et al. N Engl J Med 2016;375:1221
Khamis et al. J Clin Epidemiol 2019;113:147
Cochrane Meta-Analysis
El Moheb et al. Cochrane Database Systematic Reviews 2018
EHRA Survey shortly after DANISH
Haugaa et al. Europace 2017;19:660
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
Older age limits the benefits of ICD
Saba et al. Heart Rhythm 2019;16:1065
CRT-P vs CRT-D in elderly pts with NICM
Kristensen et al. JACC Heart Fail 2019:(in press)
Risk models in DANISH
Goldberger et al. JACC 2014;63:1879
Electrical parameters are mediocre in identifying NICM
pts ar risk for sudden death
Gulati et al. JAMA 2013;309:896
Gulati et al. JAMA 2013;309:896
Halliday et al. J Am Coll Cardiol Img 2019;12:1645
Becker et al. JACC Cardiovasc Imag 2018;11:1274
Meta-analysis of LGE as predictor of ventricular
arrhythmias or LV remodeling in DCM
Ongoing randomized trial
• 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
Di Marco et al. J Am Coll Cardiol HF 2017;5:28
Meta-analysis of LGE as predictor of SD or
ventricular arrhythmias in DCM
”Non-ischemic” cardiomyopathy
• Acromegaly
• AL amyloidosis
• Alcoholic
• Anthracycline
• Arrhythmogenic LV
• Chagas’ disease
• “Burn out” HCM
• Cocaine/metamphetamine
• Genetic/familial dilated
• Hemochromatosis
• Inflammatory/autoimmune
• PVC-induced
• LBBB-induced
• LV non-compaction
• Muscular dystrophies
• Pacing-induced
• Peripartum
• Post myocarditis
• Sarcoidosis
• Tachycardia-induced
Kayvanpour et al. Clin Res Cardiol 2017:106:135
Genetic causes of dilated cardiomyopathy
Peters et al. Heart Lung Circ 2019;28:31
Correlation between mutated genes and
cardiomyopathy phenotype
Wahbi et al. Circulation 2019;140:293
Prognostic score for pts with lamin mutations
Ortiz-Genga et al. JACC 2016:68:2440
Truncating filamin C (FLCN) mutations carry
high risk of sudden cardiac death
Gacita & McNally. Circ Heart Fail 2019;12:e005850
Proposed algorithm for high-risk mutations
Cleland et al. JACC 2017:70:1028
Reclassification of Risk of Sudden Death
My approach to ICD in nonischemic cardiomyopathy
Familial DCM with SCD or mutation with high
risk of sudden death (LMNA, SCN5A, RBM20,
FLCN)
Significant LGE in CRM?
Treatable condition?
Low risk of competing mortality?
(younger age, no severe HF (or transplant
listed), no comorbidities)
Detailed clinical and family history, deep phenotyping (CMR +), genotyping
(ie, MOGE(S) classification, JACC 2014;64:304)
ICD
ICD
No ICD, treat
and reevalaute
ICD
Prabhu et al. J Am Coll Cardiol 2017;70:1949
Prabhu et al. J Am Coll Cardiol EP 2018;4:999
AF ablation and LV fibrosis: The CAMERA-
MRI Study
Leyva et al. J Am Coll Cardiol 2012;60:1659
Patients with NICM and no fibrosis have excellent
long-term prognosis with CRT-P alone
Saini et al. JACC Clin Electrophysiol 2016;2:307
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
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.

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ICDs in nonischemic cardiomyopathy

  • 1. When to consider an ICD in the non-ischemic cardiomyopathy patient without arrhythmia? Sergio L. Pinski, MD, FHRS Cleveland Clinic Florida Weston, FL, USA
  • 2. SCD-HeFT and subsequent meta-analysis Bardy et al. N Engl J Med 2005;352:2251 Desai et al. JAMA 2004;292:2874
  • 3. Declining risk of sudden death in NICM Kober et al. N Engl J Med 2017;377:41
  • 4. The DANISH study Kober et al. N Engl J Med 2016;375:1221
  • 5. Khamis et al. J Clin Epidemiol 2019;113:147
  • 6. Cochrane Meta-Analysis El Moheb et al. Cochrane Database Systematic Reviews 2018
  • 7. EHRA Survey shortly after DANISH Haugaa et al. Europace 2017;19:660
  • 8. 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
  • 9. Elming et al. Circulation 2017;136:1772 Older age limits the benefits of ICD
  • 10. Saba et al. Heart Rhythm 2019;16:1065 CRT-P vs CRT-D in elderly pts with NICM
  • 11. Kristensen et al. JACC Heart Fail 2019:(in press) Risk models in DANISH
  • 12. Goldberger et al. JACC 2014;63:1879 Electrical parameters are mediocre in identifying NICM pts ar risk for sudden death
  • 13. Gulati et al. JAMA 2013;309:896
  • 14. Gulati et al. JAMA 2013;309:896
  • 15. Halliday et al. J Am Coll Cardiol Img 2019;12:1645
  • 16. Becker et al. JACC Cardiovasc Imag 2018;11:1274 Meta-analysis of LGE as predictor of ventricular arrhythmias or LV remodeling in DCM
  • 17. Ongoing randomized trial • 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
  • 18. Di Marco et al. J Am Coll Cardiol HF 2017;5:28 Meta-analysis of LGE as predictor of SD or ventricular arrhythmias in DCM
  • 19.
  • 20. ”Non-ischemic” cardiomyopathy • Acromegaly • AL amyloidosis • Alcoholic • Anthracycline • Arrhythmogenic LV • Chagas’ disease • “Burn out” HCM • Cocaine/metamphetamine • Genetic/familial dilated • Hemochromatosis • Inflammatory/autoimmune • PVC-induced • LBBB-induced • LV non-compaction • Muscular dystrophies • Pacing-induced • Peripartum • Post myocarditis • Sarcoidosis • Tachycardia-induced
  • 21. Kayvanpour et al. Clin Res Cardiol 2017:106:135 Genetic causes of dilated cardiomyopathy
  • 22. Peters et al. Heart Lung Circ 2019;28:31 Correlation between mutated genes and cardiomyopathy phenotype
  • 23. Wahbi et al. Circulation 2019;140:293 Prognostic score for pts with lamin mutations
  • 24. Ortiz-Genga et al. JACC 2016:68:2440 Truncating filamin C (FLCN) mutations carry high risk of sudden cardiac death
  • 25. Gacita & McNally. Circ Heart Fail 2019;12:e005850 Proposed algorithm for high-risk mutations
  • 26. Cleland et al. JACC 2017:70:1028 Reclassification of Risk of Sudden Death
  • 27. My approach to ICD in nonischemic cardiomyopathy Familial DCM with SCD or mutation with high risk of sudden death (LMNA, SCN5A, RBM20, FLCN) Significant LGE in CRM? Treatable condition? Low risk of competing mortality? (younger age, no severe HF (or transplant listed), no comorbidities) Detailed clinical and family history, deep phenotyping (CMR +), genotyping (ie, MOGE(S) classification, JACC 2014;64:304) ICD ICD No ICD, treat and reevalaute ICD
  • 28. Prabhu et al. J Am Coll Cardiol 2017;70:1949 Prabhu et al. J Am Coll Cardiol EP 2018;4:999 AF ablation and LV fibrosis: The CAMERA- MRI Study
  • 29. Leyva et al. J Am Coll Cardiol 2012;60:1659 Patients with NICM and no fibrosis have excellent long-term prognosis with CRT-P alone
  • 30. Saini et al. JACC Clin Electrophysiol 2016;2:307 CRT responders have lower risk of ventricular arrhythmia
  • 31. 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
  • 32. 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.