1 9/14/2019 The DANISH study Khamis et al. J Clin Epidemiol - - PDF document

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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

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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

Disclosures

Nothing to declare

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

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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

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Refining the indication-Risk stratification

Select patients with higher risk of sudden cardiac death but lower risk
f death due to heart failure and competing comorbidities

Elming et al. Circulation 2017;136:1772

Older age limits the benefits of ICD

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

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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

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”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

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

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Ortiz-Genga et al. JACC 2016:68:2440

Truncating filamin C (FLCN) mutations carry high risk of sudden cardiac death

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

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

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Saini et al. JACC Clin Electrophysiol2016;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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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

Kayvanpour et al. Clin Res Cardiol 2017:106:135

Genetic causes of dilated cardiomyopathy

Kristensen et al. JACC Heart Fail 2019:(in press)

Risk models in DANISH

Gacita & McNally. Circ Heart Fail 2019;12:e005850

Proposed algorithm for high-risk mutations

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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

Voscoboinik et al. PACE 2018;41:1109

Majority of “NICM” patients resuscitated from VF did not have fibrosis in CMR

Saba et al. Heart Rhythm 2019;16:1065