Table 1.
Recommendations for ICDs as Primary Prevention in Familial Cardiomyopathy
| Phenotype | Recommended consideration for ICD | Level | Comments |
|---|---|---|---|
| Hypertrophic Cardiomyopathy | Single risk factor: Family history of sudden death Massive LVH (≥ 30 mm) Unexplained syncope LVEF < 0.50 LV apical aneurysm | IIa | From 2020 AHA/ACC Guideline for HCM31 |
| NSVT or LGE ≥ 15% | IIb | ||
| Dilated Cardiomyopathy | Non-ischemic CM with EF ≤ 0.35, despite GDMT**, and NYHA II-III symptoms of HF | I | From 2017 AHA/ACC/HRS Guideline for Management of Patients with Ventricular Arrhythmias and Prevention of Sudden Cardiac Death32 |
| LVEF≤ 0.30 despite GDMT**, NYHA Class I | IIb | ||
| Specific genetic DCM when LVEF ≥ 0.35 | Lamin A/C mutation with ≥ 2 risk factors: Male, LVEF<0.45, NSVT, Non missense variant | IIa | |
| Lamin A/C ≥ 2 risk factors Male sex, LVEF < 0.45, NSVT | IIa | From 2019 HRS Expert Consensus Statement on Evaluation, Risk stratification, and Management of Arrhythmogenic Cardiomyopathy2 | |
| Lamin A/C and pacing indication | IIa | ||
| Phospholamban LVEF < 0.45 or NSVT | IIa | ||
| Filamin C LVEF < 0.45 | IIa | ||
| Arrhythmogenic RV CM (ARVC) | (Resuscitated SCA, sustained VT*) or significant ventricular dysfunction with RVEF or LVEF ≤ 0.35 | I | From 2017 AHA/ACC/HRS Guideline32 |
| 3 major criteria, 2 major+ 2 minor, 1 major+ 4 minor criteria | IIa | From 2019 HRS Expert Consensus Statement2 Major criteria: NSVT, inducible VT, LVEF ≤ 0.49 Minor criteria: Male sex, > 1000 PVCs/24 hours, RV dysfunction by 2010 criteria33, proband status, ≥ 2 variants (cannot count both NSVT and PVCs.) | |
| 2 major, 1 major+2 minor criteria, or 4 minor criteria | IIb | ||
| LV Non- Compaction | NSVT associated with reduced LVEF | IIa | From 2019 HRS Expert Consensus Statement2 |
| Neuromuscular disorders | Emery-Dreifuss and limb-girdle type IB dystrophies with progressive cardiac involvement | IIa | From 2017 AHA/ACC/HRS Guideline32 |
|
Examples of variants that can cause ACM for which there is no recommendation when LVEF ≥ 0.35
RNA-binding motif 20 (RBM20) Type V voltage-gated cardiac sodium channel (SCN5A) Desmoplakin (DSP) ACM not meeting criteria for ACM-RV | |||
| Familial CM unspecified | Familial cardiomyopathy associated with sudden death | IIb | From ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities34, and 2013 Appropriate Use Criteria for ICDs and CRTs35 |
*
Syncope and sustained ventricular tachycardia are considered to be secondary prevention
**
GDMT = guideline-directed medical therapy for at least 3 months