Neuromuscular diseases (NMD) encompass a broad spectrum of diseases with 
variable type of cardiac involvement and there is lack of clinical data on 
Cardiovascular Magnetic Resonance (CMR) phenotypes or even prognostic value of 
CMR in NMD. We explored the diagnostic and prognostic value of CMR in 
NMD-related cardiomyopathies. The study included retrospective analysis of a 
cohort of 111 patients with various forms of NMD; mitochondrial: n = 14, 
Friedreich's ataxia (FA): n = 27, myotonic dystrophy: n = 27, Becker/Duchenne's 
muscular dystrophy (BMD/DMD): n = 15, Duchenne's carriers: n = 6, other: n = 22. 
Biventricular volumes and function and myocardial late gadolinium enhancement 
(LGE) pattern and extent were assessed by CMR. Patients were followed-up for the 
composite clinical endpoint of death, heart failure development or need for 
permanent pacemaker/intracardiac defibrillator. The major NMD subtypes, i.e. FA, 
mitochondrial, BMD/DMD, and myotonic dystrophy had significant differences in 
the incidence of LGE (56%, 21%, 62% & 30% respectively, chi2 = 9.86, p = 0.042) 
and type of cardiomyopathy phenotype (chi2 = 13.8, p = 0.008), extent/pattern 
(p = 0.006) and progression rate of LGE (p = 0.006). In survival analysis the 
composite clinical endpoint differed significantly between NMD subtypes 
(p = 0.031), while the subgroup with LGE + and LVEF < 50% had the worst 
prognosis (Log-rank p = 0.0034). We present data from a unique cohort of NMD 
patients and provide evidence on the incidence, patterns, and the prognostic 
value of LGE in NMD-related cardiomyopathy. LGE is variably present in NMD 
subtypes and correlates with LV remodelling, dysfunction, and clinical outcomes 
in patients with NMD.