Cardiac magnetic resonance (CMR) has become an essential tool for the evaluation of patients affected or at risk of developing cardiomyopathies (CMPs). In fact, CMR not only provides precise data on cardiac volumes, wall thickness, mass and systolic function but it also a non-invasive characterization of myocardial tissue, thus helping the early diagnosis and the precise phenotyping of the different CMPs, which is essential for early and individualized treatment of patients. Furthermore, several CMR characteristics, such as the presence of extensive LGE or abnormal mapping values, are emerging as prognostic markers, therefore helping to define patients’ risk. Lastly new experimental CMR techniques are under investigation and might contribute to widen our knowledge in the field of CMPs. In this perspective, CMR appears an essential tool to be systematically applied in the diagnostic and prognostic work-up of CMPs in clinical practice. This review provides a deep overview of clinical applicability of standard and emerging CMR techniques in the management of CMPs.

Aims: Left ventricular (LV) ring-like scar on cardiac magnetic resonance (CMR) has been linked to malignant arrhythmias in patients with non-ischaemic cardiomyopathy. This study aimed to perform a comprehensive evaluation of this phenotype and to identify risk factors for life-threatening arrhythmic events (LAEs), a composite of sudden cardiac death (SCD), aborted SCD, and sustained ventricular tachycardia. Methods and results: One hundred and fifteen patients [median age 39 (interquartile range, IQR, 28–52), 42% females] were identified at 6 referral centres. Inclusion criteria were ring-like LV scar [≥3 contiguous segments with sub-epicardial/midwall late gadolinium enhancement (LGE) in the same slice] and one among: pathogenic/likely pathogenic genetic variant, family history for cardiomyopathy, or arrhythmogenic cardiomyopathy diagnosis. During the study follow-up, survival free from LAEs was 60% (3.8 events/100 patients/year); at a median follow-up of 4.6 years (IQR 1.7–8.4) it was 84%. On multivariable analysis, anterior Q waves [hazard ratio (HR): 1.030, 95% confidence intervals (CI): 1.014–1.046, P < 0.001], QRS width (HR: 4.642, 95% CI: 1.296–16.628, P = 0.018), and LV end-diastolic volume index (LVEDVi; HR: 1.011, 95% CI: 1.001–1.021, per mL/m2 increase, P = 0.040) were independently associated with LAEs; with good discrimination power (Harrell’s C-index = 0.796). Three risk categories were identified: normal electrocardiogram (ECG), abnormal ECG and no LAEs predictive variables, abnormal ECG and ≥1 LAEs predictive variables, with a decreasing survival from 100 to 65% and 49%, respectively (Log-rank test = 0.015). Conclusion: In this study, the LV ring-like scar phenotype was associated with a high rate of malignant arrhythmias in presence of anterior Q waves, QRS prolongation, and increased LVEDVi. A normal ECG identified a lower risk sub-group.

Severe mitral valve regurgitation (MR) carries a significant burden both in prognosis and quality of life of patients, as well as on healthcare systems, with high rates of hospitalization for heart failure. While mitral valve surgery constitutes the first-line treatment option for primary MR in suitable patients, surgical treatment for secondary severe MR remains controversial, with a substantial lack of evidence on a survival benefit. In recent decades, percutaneous mitral valve repair has emerged as an alternative treatment for patients deemed not suitable for surgery. Among several devices under development or evaluation, the MitraClip system is the most widespread and is supported by the strongest evidence. While the role of MitraClip in patients with chronic primary MR who are not deemed suitable for surgery is well established, with consistent data showing improvement in both prognosis and quality of life, MitraClip treatment in secondary MR is a rapidly evolving field. Two recent randomized clinical trials generated apparently controversial results but actually provided an interesting pathophysiologic frame that could help discerning patients who will benefit from the procedure versus patients who will not. In this review, we will discuss current treatment options for mitral regurgitation, focusing on percutaneous mitral valve repair with the MitraClip system.

Cardiac magnetic resonance (CMR) imaging provides significant advantages in the non-invasive diagnosis of cardiac diseases. An emerging phenotype is increasingly being described in CMR reports, the LGE “ring-like” pattern, which resembles a circumferential/semi-circumferential LV scar. Different conditions exhibit this fibrosis distribution, the majority of them being genetically determined and mostly involving cardiomyopathy-causative genes (desmosomal but also other non-desmosomal related genes). Furthermore, inflammatory diseases, such as myocarditis or sarcoidosis, could be responsible for LV fibrosis, potentially exhibiting an RL distribution. Given the heterogeneity of such conditions, effective patient management requires a stepwise and multiparametric diagnostic work-up that integrates clinical, instrumental, and genetic data to identify the specific aetiology and guide personalised treatments.

Background: Cardiac involvement in Anderson-Fabry disease (AFD) is related to a progressive glycosphingolipid storage over time and is characterized by left ventricular hypertrophy (LVH), conduction abnormalities and myocardial fibrosis. ECG is useful for early recognition of AFD, however evidence is limited on the association between ECG alterations and disease stage. Purpose: To assess the relationship between ECG characteristics and progressive cardiac involvement, from the pre-hypertrophic phase to phenotypes with increasing degree of LVH. Methods: In a multicenter cohort, 183 AFD patients (40% male, age 47±12 years, 60% affected by “classical AFD”) underwent ECG and transthoracic echocardiography. Patients were divided into 4 groups according to the different degree of LV thickness measured in parasternal short axis view: group A ≤9 mm (N=46, 25%), group B 10–14 mm (N=77, 42%), group C 15–19 mm (N=45, 25%) and group D ≥20 mm (N=15, 8%). Patients with pacemaker and under 18 years of age were excluded. Results: A normal ECG was present in 89% in group A, 59% in group B, 11% in group C and it was absent in group D. Short PR (<120 ms) was more frequent in group A, whereas with LVH increasing, median PR interval duration significantly prolonged among the 4 groups (136 [125–150]vs 141 [130–160] vs 160 [130–180] vs 170 [130–180] ms, p=0.002 respectively). Median P-wave duration was shorter in group A and B compared to group C and D (80 m vs 100 ms, p<0.001), while both QRS and QTc gradually increased. Median Sokolow-Lyon voltage criteria statistically augmented among the groups (22 [18–26] vs 27 [20–33] vs 32 [25–45] vs 35 [18–40] mm, p<0.001 respectively), along with right ventricular hypertrophy (0%, 1%, 11%, 8%, p=0.02). Right bundle branch block (RBBB) had a higher frequency in advanced stages (0%, 34%, 34%, 40%, p<0.001), with a prevalence of complete RBBB of 46% in group D. Similarly, left anterior fascicular block (0%, 7%, 18%, 46%, p<0.001) and QRS fragmentation (2%, 11%, 25%, 23%, p=0.009) were more common in advanced stages. No differences were found in left bundle branch block (LBBB), in low QRS voltages or in LV pre-excitation prevalence. According with the wall thickness increase, negative T waves were more frequent in lateral (4%, 21%, 70%, 77%, p<0.001) and inferior leads (6%, 15%, 32%, 46%, p 0.001), as well as their association with ST-T depression (4%, 17%, 64%, 46%, p<0.001). Giant negative T waves were present only in group C and D (16% and 31%) mainly representing a LVH distribution toward the apex. Conclusions: ECG is a very useful tool to stage cardiac involvement evolution in AFD. Peculiar ECG characteristics evolve together with LV wall thickness: incomplete and progressively complete RBBB usually associated (preceding or following) LVH and/or typical repolarization abnormalities in inferior or lateral leads and giant negative T waves in the more advanced stages are the most frequent and typical ECG patterns. Funding Acknowledgement: Type of funding sources: None.