Presented at 10th California Heart Rhythm Symposium, San Francisco, Sep 2019

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

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.