RCT of Implantable Defibrillators in Patients with Non Ischemic Cardiomyopathy, Scar and Severe Systolic Heart Failure

Inclusion Criteria:

1. A diagnosis of NICM on contrast-enhanced cardiovascular magnetic resonance imaging

2. LV scar on routine CMR (patient without scar can enter the registry)

3. New York Heart Association (NYHA) Heart Failure (HF) functional class I-III andseverely impaired left ventricular function (LVEF ≤ 35% on any imaging modality)after a minimum of 3 months of treatment with optimal medical therapy (OMT) asrecommended by National Institute for Health and Care Excellence (NICE)

4. Able and willing to provide informed consent

Exclusion Criteria:

1. New York Heart Association (NYHA) HF functional class IV after 3 months of optimalmedical therapy (OMT)

2. Acute decompensated heart failure

3. Previous implantable device in situ (PPM, Cardiac Resynchronisation Therapy (CRT) orICD)

4. Ischemic cardiomyopathy (ICM) is defined as segmental wall motion abnormalities orwall thinning in a particular coronary territory with subendocardial or transmurallate gadolinium enhancement (LGE). Patients with an LVEF ≤35% and a small amount ofischemic LGE (i.e. an infarct out of keeping with the amount of LV dysfunction) willnot be excluded (so-called dual pathology patients)

5. Known diagnosis of amyloidosis, sarcoidosis, arrhythmogenic right ventricularcardiomyopathy, or hypertrophic cardiomyopathy (diseases in which there are specificguidelines regarding defibrillator therapy)

6. Known Lamin gene mutation or a positive family history of a Lamin gene mutation

7. Valve disease is considered likely to require surgery during the 3 years follow-upperiod

8. Complex congenital heart disease

9. Any secondary prevention ICD indication

10. Heart transplant recipient or admitted for cardiac transplantation/ left ventricularassist device

11. Clinically apparent myocardial ischemia which requires revascularization

12. Intracardiac mass which requires surgery

13. Active endocarditis

14. Active Septicaemia

15. Pregnancy

16. Life expectancy <2 years secondary to any other cause (i.e. malignancy)

17. Active treatment with chemotherapy

18. Severe renal failure (GFR <30)

19. Actively participating in another study without prior agreement between both ChiefInvestigators