Truncating mutations in FLNC caused an overlapping phenotype of dilated and left-dominant arrhythmogenic cardiomyopathies complicated by frequent premature sudden death. Prompt implantation of a cardiac defibrillator should be considered in affected patients harboring truncating mutations in FLNC.

In patients with AM and preserved LVEF, LGE in the midwall layer of the AS myocardial segment is associated with a worse prognosis than other patterns of presentation.

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The presence of a wide and/or large S-wave in lead I was a powerful predictor of life-threatening ventricular arrhythmias in patients with BrS and no history of cardiac arrest at presentation. However, the prognostic value of a significant S-wave in lead I should be confirmed by larger studies and by an independent confirmation cohort of healthy subjects.

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Aims The aim of this registry was to evaluate the additional prognostic value of a composite cardiac magnetic resonance (CMR)-based risk score over standard-of-care (SOC) evaluation in a large cohort of consecutive unselected non-ischaemic cardiomyopathy (NICM) patients. Methods and results In the DERIVATE registry (www.clinicaltrials.gov/registration: RCT#NCT03352648), 1000 (derivation cohort) and 508 (validation cohort) NICM patients with chronic heart failure (HF) and left ventricular ejection fraction <50% were included. All-cause mortality and major adverse arrhythmic cardiac events (MAACE) were the primary and secondary endpoints, respectively. During a median follow-up of 959 days, all-cause mortality and MAACE occurred in 72 (7%) and 93 (9%) patients, respectively. Age and >3 segments with midwall fibrosis on late gadolinium enhancement (LGE) were the only independent predictors of all-cause mortality (HR: 1.036, 95% CI: 1.0117–1.056, P < 0.001 and HR: 2.077, 95% CI: 1.211–3.562, P = 0.008, respectively). For MAACE, the independent predictors were male gender, left ventricular end-diastolic volume index by CMR (CMR-LVEDVi), and >3 segments with midwall fibrosis on LGE (HR: 2.131, 95% CI: 1.231–3.690, P = 0.007; HR: 3.161, 95% CI: 1.750–5.709, P < 0.001; and HR: 1.693, 95% CI: 1.084–2.644, P = 0.021, respectively). A composite clinical and CMR-based risk score provided a net reclassification improvement of 63.7% (P < 0.001) for MAACE occurrence when added to the model based on SOC evaluation. These findings were confirmed in the validation cohort. Conclusion In a large multicentre, multivendor cohort registry reflecting daily clinical practice in NICM work-up, a composite clinical and CMR-based risk score provides incremental prognostic value beyond SOC evaluation, which may have impact on the indication of implantable cardioverter-defibrillator implantation.

Accumulation of fat at ectopic sites has been gaining attention as pivotal contributor of insulin resistance, metabolic syndrome and related cardiovascular complications. Intermuscular adipose tissue (IMAT), located between skeletal muscle bundles and beneath muscle fascia, has been linked to physical inactivity, ageing and body mass index, but little is known about its relationship with the other AT compartments, in particular with increasing age. To address this issue, erector spinae IMAT, epicardial (EAT), intraabdominal (IAAT) and abdominal subcutaneous adipose tissue (SAT) were simultaneously measured by Magnetic Resonance Imaging (MRI) and related to waist circumference measurements and age in 32 sedentary subjects without cardiovascular disease (18 men; 14 women; mean age 48.5±14 years). Fasting glucose, triglycerides and HDL-cholesterol were also assessed. We observed that, after dividing individuals according to age (≤ or >50 years), IMAT and EAT depots were significantly more expanded in older subjects (63.2±8.3 years) than in the younger ones (38.4±5.2 years) (p<0.001). Overall, both IMAT and EAT showed stronger positive associations with increasing age (β = 0.63 and 0.67, respectively, p<0.001 for both) than with waist circumference (β = 0.55 and 0.49, respectively, p<0.01 for both) after adjusting for gender. In addition, the gender-adjusted associations of IMAT and EAT with waist circumference and IAAT were significant in individuals ≤50 years only (p<0.05 for all) and not in the older ones. In contrast, no age-related differences were seen in the relationships of IAAT and SAT with waist circumference. Finally, serum triglycerides levels turned out not to be independently related with ectopic IMAT and EAT. In conclusion, the expansion of IMAT and EAT in sedentary subjects is more strongly related to age than waist circumference, and a positive association of these ectopic depots with waist circumference and IAAT amount can be postulated in younger individuals only.

Background:The best timing for coronary angiography (immediate vs early) in patients with acute non-STelevation myocardial infarction (NSTEMI) is controversial. Hypothesis: Evaluate in NSTEMI patients the effects of an immediate compared to an early invasive strategy on microvascular damage, myocardial perfusion, and infarct size. Methods: We randomized 54 consecutive patients with first episode of NSTEMI: 27 patients (22 males, age 58.8 ± 9.4 years, group A) underwent immediate (≤6 hours) percutaneous coronary intervention (PCI) with a double bolus of eptifibatide, and 27 patients (24 males, age 59.7 ± 9.8 years, P = 0.72, group B) underwent early (7-72 hours) PCI with upstream eptifibatide. Microvascular damage was evaluated at predischarge by myocardial contrast echocardiography, and the contrast defect length was calculated.Results: There were no significant differences in pre-PCI myocardial blush grade (MBG) (41% MBG 0 or 1 in group A vs 37% MBG 0 or 1 in group B, P = 0.78), in post-PCI MBG (7.4% MBG 0 or 1 in both groups, P = 1.00), and in contrast defect length (4.5% in group A vs 2.8% in group B, P = 0.56). However, group A showed a significant reduction in creatine kinase myocardial band isoenzyme peak (26 ± 26 ng/mL in group A vs 69 ± 79 ng/mL in group B, P = 0.01) and in troponin T peak (0.84 ± 1.2 ng/mL in group A vs 1.8 ± 2.1 ng/mL in group B, P = 0.048). Conclusions:In patients with NSTEMI treated with eptifibatide, immediate PCI is associated with less increase in myonecrosis markers compared with PCI within 72 hours. There were no significant differences in myocardial perfusion between the 2 strategies. IntroductionIn patients with acute non-ST-elevation myocardial infarction (NSTEMI), an early invasive strategy with coronary angiography (CA) and subsequent revascularization through percutaneous coronary intervention (PCI) is associated with reduced rates of death and myocardial infarction (MI) in comparison with a conservative strategy. 1 Guidelines for the treatment of patients with acute coronary syndromes (ACS) recommend an early (≤72 hours) invasive strategy for patients admitted with diagnosis of NSTEMI. 2 Excluding conditions requiring emergency interventions (refractory angina, shock, and life-threatening arrhythmias), controversy remains regarding the optimal timing between hospital admission and the invasive procedure, with 2 different alternatives for the invasive approach: