Cardiovascular magnetic resonance in neuromuscular disorders: looking ahead

Abstract Funding Acknowledgements Type of funding sources: None. Background Neuromuscular disorders (NMD) have a wide range different cardiac presentations. Cardiac magnetic resonance (CMR) has an established role in diagnosis and risk stratification. We sought to access how CMR performs predicting events real cohort NMD patients (pts). Methods included consecutive pts followed tertiary clinical center with from January 2012 December 2018. Clinical data were collected. During follow-up (FUP), we considered major adverse cardiovascular (MACE) as composite device implantation, ventricular tachycardia/appropriate shock therapy death. Results A total 65 (pts) included, 33 (51%) women, mean age 32 ± 16 years. Most had myotonic dystrophy (34, 52%), by limb–girdle muscular (22; 34%); the remained 9 (13%) other NMD. About half inferior limbs predominantly affected 74% none, mild or moderate functional impairment. Regarding manifestations, 18% symptoms, 97% sinus rhythm, median PR QRS duration 169 (IQR 47) ms 101 11) ms, respectively; BNP was 26 25) mg/dl. CMR, 43,3% ≥ one abnormality. Six left ventricle dilation 7 ejection fraction (LVEF) 55%. Three significant hypertrophy (>12 mm) there isolated cases hypertrabeculation, segmental alterations right dilation. tissue characterization, 2 T2 hyperintensity, 8 early gadolinium enhancement (EGE) 22 late (LGE). LGE located mainly intramyocardium (45%) subepicardial (36%) most segments basal medium inferolateral (40%). FUP 77 33) months deaths, implanted devices (4 pacemakers 4 CRT-D, 3 primary prevention) sustained tachycardia holter; no therapies. Table 1 describes some parameters according occurrence events. Using Kaplan Meier curves, associations between LVEF<55% presence allevents (log rank test, p = 0,002 0,045, respectively), but association found age, pattern nor number/distribution segments. Cox Regression, that associated 6 fold higher (HR crude 6,15; 95% CI 1,65–22,93), after adjusting for adjusted 4,81, 1,07–15,9). Conclusion In our cohort, significantly during FUP, reinforcing this technique on stratification populations