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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

Aims Eosinophilic heart disease (EHD) is a rare cardiac condition with a wide spectrum of phenotypes. The diagnostic and prognostic value of cardiac magnetic resonance (CMR) in EHD remains unknown. Methods and results This was a retrospective analysis of 250 patients with eosinophilia referred for a CMR scan (period 2000–2020). CMR data sets and clinical/laboratory data were collected. Patients were followed up for a mean of 24 months (range 1–224) for the composite endpoint of death, acute coronary syndrome, hospitalization for acute heart failure, malignant ventricular arrhythmias, or the need for implantable cardiac defibrillator/pacemaker. The main objectives were to explore the diagnostic value of CMR in EHD; relationships between cardiac function, late gadolinium enhancement (LGE), and EHD phenotypes; and the prognostic value of fibrosis and oedema by CMR. The prevalence of findings compatible with EHD was 39% (patients with cardiac symptoms: 57% vs. screening: 20%, P Conclusion The diagnostic yield of CMR screening in patients with persistent eosinophilia, even if asymptomatic, is high. The extent of subendocardial fibrosis correlates with LV remodelling and independently predicts clinical outcomes in patients with eosinophilia.

Background This is a case report of a primary cardiac lymphoma with an unusual clinical presentation. We hereby illustrate the characteristic features of cardiac lymphomas by multimodality imaging and particularly cardiac magnetic resonance (CMR) that can help reach a timely diagnosis non-invasively and guide treatment decisions. Case summary A 58-year-old woman, without significant past medical history, presented with a 3-week history of shortness of breath associated with facial and neck swelling. Transthoracic echocardiogram confirmed the presence of a cardiac mass in the right atrium. Cardiac magnetic resonance helped to characterize the mass, assess its haemodynamic significance and relation to cardiac structures, and reach a non-invasive diagnosis that was crucial for guiding treatment decisions and interventions. Discussion Cardiac masses have distinct imaging features that can help differentiate malignant from benign cardiac tumours. More specifically, primary cardiac lymphomas can be relatively easy diagnosed by CMR in most cases thanks to their characteristic imaging appearance.

BACKGROUND: Right ventricular (RV) failure post-pericardiectomy for constrictive pericarditis (CP) has been reported but it remains not well-studied. To investigate imaging parameters that could predict RV function and the outcome of patients post-pericardiectomy. METHODS: We analysed data from a total of 53 CP patients undergoing pericardiectomy. Preoperative, early and at 6 months postoperative echocardiographic (echo) imaging datasets were analysed and correlated with preoperative cardiac magnetic resonance (CMR), cardiac computed tomography scans and histology. The primary endpoint of the study was RV functional status early postoperatively and at 6 months. Secondary endpoint was the need for prolonged inotropic support. RESULTS: A cause of CP was identified in 26 patients (49%). Inotropic support ≥ 48 hours was required in n = 28 (53%) of patients and was correlated with lower preoperative RV areas by echo or RV volumes by CMR (p < 0.05 for all). A pericardial score based on pericardial thickness/calcification and epicardial fat thickness had good diagnostic accuracy to identify patients requiring prolonged use of inotropes (area under the curve, 0.825; 95% confidence interval, 0.674–0.976). Pericardiectomy resulted in RV decompression and impaired RV function early postoperatively (fractional area change: 40.5% ± 8.8% preoperatively vs. 31.4% ± 10.4% early postoperatively vs. 42.5% ± 10.2% at 6 months, p < 0.001). CONCLUSIONS: We show that a smaller RV cavity size and a pericardial scoring system are associated with prolonged inotropic support in CP patients undergoing pericardiectomy. RV systolic impairment post decompression is present in most patients, but it is only transient.

We report an unusual clinical case of a 66-year-old patient with cardiac involvement from a metastatic melanoma, causing the formation of a large right atrial mass with extensive infiltration of the right atrial free wall, the interatrial septum, the coronary sinus, and up to the mitral annulus and posterior wall of the right ventricle, unamendable to complete surgical excision. As secondary cardiac tumors are not part of routine daily clinical practice, we thought that this clinical case would be a good educational opportunity for the practicing clinicians, both specialists and nonspecialists.