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ObjectiveCardiac sarcoidosis (CS) may present with cardiac arrest or life-threatening arrhythmias. There are limited data on this subgroup of patients with CS. Advanced imaging including cardiovascular magnetic resonance (CMR) and cardiac 18-fluorodeoxyglucose (FDG) positron emission tomography (PET) are used for diagnosis. This study aimed to describe advanced imaging patterns suggestive of CS among patients presenting with cardiac arrest or life-threatening arrhythmias.MethodsAn imaging database of a CS referral centre (Royal Brompton Hospital, London) was screened for patients presenting with cardiac arrest or life-threatening arrhythmias and having imaging features of suspected CS. Patients diagnosed with definite or probable/possible CS were included.ResultsStudy population included 60 patients (median age 49 years) with male predominance (76.7%). The left ventricle was usually non-dilated with mildly reduced ejection fraction (53.4±14.8%). CMR studies showed extensive late gadolinium enhancement (LGE) with 5 (4–8) myocardial segments per patient affected; the right ventricular (RV) side of the septum (28/45) and basal anteroseptum (28/45) were most frequently involved. Myocardial inflammation by FDG-PET was detected in 45 out of 58 patients vs 11 out of 33 patients with oedema imaging available on CMR. When PET was treated as reference to detect myocardial inflammation, CMR oedema imaging was 33.3% sensitive and 77% specific.ConclusionsIn patients with CS presenting with cardiac arrest or life-threatening arrhythmias, LGE was located in areas where the cardiac conduction system travels (basal anteroseptal wall and RV side of the septum). While CMR was the imaging technique that raised possibility of cardiac scarring, oedema imaging had low sensitivity to detect myocardial inflammation compared with FDG-PET.

Reversal of torrential tricuspid regurgitation is rarely seen. We describe a case in which effective immunosuppression alongside conventional heart failure therapies lead to reversibility of torrential tricuspid regurgitation in a patient with cardiac sarcoidosis. We also discuss the diagnostic challenge in distinguishing cardiac sarcoidosis from other myocardial diseases in a patient presenting with biventricular failure.

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.

Aims Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. Methods and results The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (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), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. Conclusions 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.

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.

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, chi

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.