Arrhythmogenic cardiomyopathy (ACM) is a genetically determined heart muscle disease characterized by fibro-fatty myocardial replacement, clinically associated with malignant ventricular arrhythmias and sudden cardiac death. Originally described a disease with a prevalent right ventricular (RV) involvement, subsequently two other phenotypes have been recognized, such as the left dominant and the biventricular phenotypes, for which a recent International Expert consensus document provided upgrade diagnostic criteria (the 2020 “Padua Criteria”). In this novel workup for the diagnosis of the entire spectrum of phenotypic variants of ACM, including left ventricular (LV) variants, cardiac magnetic resonance (CMR) has emerged as the cardiac imaging technique of choice, due to its capability of detailed morpho-functional and tissue characterization evaluation of both RV and LV. In this review, the key role of CMR in the diagnosis of ACM is outlined, including the supplemental value for the characterization of the disease variants. An ACM-specific CMR study protocol, as well as strengths and weaknesses of each imaging technique, is also provided. Key Points • Arrhythmogenic cardiomyopathy includes three different phenotypes: dominant right, biventricular, and dominant left. • In 2020, diagnostic criteria have been updated and cardiac magnetic resonance has emerged as the cardiac imaging technique of choice. • This aim of this review is to provide an update of the current state of art regarding the use of CMR in ACM, with a particular focus on novel diagnostic criteria, CMR protocols, and prognostic significance of CMR findings in ACM.

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Background The 2020 severe acute respiratory syndrome coronavirus 2 outbreak entailed reduced availability of traditional (in-office) cardiology consultations. Remote monitoring is an alternative way of caring that may potentially mitigate the negative effects of the epidemic to the care of cardiovascular diseases. We evaluated the outcome of implantable cardioverter defibrillator (ICD) carriers followed up remotely in 2020 (epidemic period) versus 2019 (control). Methods We included all patients with an ICD who remained remotely monitored from the beginning to the end of each year. The combined end point included: new-onset atrial fibrillation; sustained ventricular tachycardia >170 bpm without ICD intervention; appropriate ICD intervention (either shock or antitachycardia pacing); any-cause death. Multiple events in the same patients were counted separately if occurring ≥48 h apart. Results In 2020, 52 end points occurred in 37 of 366 (10%) ICD carriers [0.14/patient (95% confidence interval [CI] = 0.11–0.19)] versus 43 end points in 32 of 325 (10%) ICD carriers in 2019 [0.13/patient (95% CI = 0.10–0.18) P = 0.75]. There was no difference between the distribution of any individual end point in 2020 versus 2019 although a nonsignificant mortality increase was observed (from 2.8% to 4.6%, P = 0.19). The lowest weekly event rate occurred during the national lock down in spring 2020 but a similar trend occurred also in 2019 suggesting that the effect may not be linked to social distancing measures. Conclusions We did not observe an increase in a combined end point including arrhythmic events and mortality in ICD carriers who were remotely monitored in 2020, compared to 2019, despite the negative impact of the coronavirus disease 2019 outbreak on the healthcare system.

Background Low-QRS voltages (LQRSV) are an unexpected finding in left ventricular (LV) hypertrophy, i.e, hypertrophic cardiomyopathy (HCM) or athlete’s heart. Methods Prevalence and clinical correlates of LQRSV were investigated in 197 consecutive HCM patients, aged 58±13 years and comparatively in 771 Olympic athletes, aged 23± 4. Clinical characterization included family/personal history, symptoms, NYHA functional class, ECG pattern, ventricular arrhythmias and cardiac magnetic resonance (CMR). Results 22 (11%) of HCM, and 18 (2.3%) of athletes presented LQRSV. At initial evaluation, in HCM, LQRSV showed no differences vs. non-LQRSV for functional class (90% vs. 91%, in class I-II; p=0.983), symptoms (27% vs. 18%; p=0.478), ventricular arrhythmias (40% vs. 39%; p=857), but showed larger extent of LGE at CMR (4.1±1.5 vs. 1.5±0.7 affected segments; p<0.001). In athletes, LQRSV was associated with larger prevalence of inverted T-waves (22% vs. 9%; p<0.001) and ventricular arrhythmias (28% vs. 8%; p=0.005). In one LQRSV athlete arrhythmogenic cardiomyopathy was identified. Over 4.5±2.6-year follow-up, presence of LQRSV in HCM was associated with larger incidence of functional deterioration (31% vs. 14%; p=0.038); stroke (22% vs. 6%; p=0.008) and ICD implant (27% vs. 10%; p=0.015). No clinical events occurred in LQRSV athletes without initial evidence of cardiac disease. Conclusion LQRSV are relatively common (11%) in HCM and have clinical relevance, being predictive over a medium-term for a worsening functional class, incidence of stroke and ICD implant. Instead, LQRSV are rare (2.3%) in athletes, but may occasionally be a marker that raises suspicion for underlying cardiac disease at risk.