Abstract Background The ability of echocardiography (TTE) to diagnose cardiac sarcoidosis (CS) has traditionally been limited by its low sensitivity. We sought to determine the optimal TTE parameters to detect cardiac involvement in sarcoidos is with the inclusion of strain and 3DE to standard conventional TTE data. Methods Consecutive patients referred for evaluation of suspected CS were prospectively recruited and underwent symptom evaluation, 12-lead ECG, ambulatory ECG monitoring, TTE with 3DE and strain, cardiac magnetic resonance and 18F-FDG-PET. We identified the TTE phenotype most likely to identify CS. We evaluated the ability of a novel multi-parametric approach to screen for cardiac involvement among sarcoidosis patients and compared this to existing criteria. Results Of the 181 patients recruited (mean age 55 ± 11 years, 60% male, mean LVEF 57 ± 10%), 106 (59%) were diagnosed with CS. The strongest TTE parameters to predict CS were the presence of a dilated LV cavity (end-diastolic/end-systolic ≥ 71/28ml/m2 females; ≥80/32ml/m2 males), LVEF < 50%, RWMA involving multiple myocardial territories and basal septal thinning. This ‘probable CS’ TTE model had 96% specificity, 33% sensitivity and 92% PPV for the diagnosis of CS. The strain parameters most specific for a CS diagnosis were LVGLS and LVGCS greater than − 13% and − 15%, respectively. Adding strain or 3D data did not significantly improve the overall diagnostic ability of TTE. The optimal screening strategy for CS among sarcoidosis patients involved evaluation of symptoms, ECG or ambulatory ECG monitoring, and ‘probable CS’ TTE. This approach had a sensitivity of 90%, compared to 77% and 84% for the 2014 Heart Rhythm Society and 2020 American Thoracic Society criteria, respectively. In the asymptomatic patient, the absence of abnormal rhythm data and a normal TTE excluded CS in 94% of patients. Conclusion The presence of LV dilatation, LV systolic impairment, multi-territory RWMA and basal septal thinning are the most specific TTE parameters for the detection of CS. In combination with symptom evaluation and ECG data, comprehensive echocardiography remains a useful screening tool among sarcoidosis patients with suspected cardiac involvement.

Inflammatory cardiac diseases such as myocarditis, cardiac sarcoidosis, vasculitis, and immune-mediated toxicities remain diagnostically challenging because clinically relevant questions often concern whether inflammation is active, resolving, or already caused a scar. Positron emission tomography (PET) has moved from a predominantly fluorodeoxyglucose ([18F]FDG)-based approach toward a broader molecular imaging platform that combines technical innovation with tracer diversification. This review summarizes recent advances in PET imaging of inflammatory cardiac disease, including new hardware and promising new radiotracers. Current evidence supports PET as a tool not only for diagnosis but also for treatment monitoring and risk stratification, and future progress will depend on standardization, histologic validation, and integration of advanced hardware, disease-specific tracers, and multimodality biomarkers into clinically actionable pathways.

Primary cardiac lymphomas (PCLs) are rare and often present a diagnostic challenge because of nonspecific clinical and imaging findings. Delayed diagnosis may lead to rapid clinical deterioration. We report a case series of 6 patients with PCL diagnosed between 2021 and 2025 at 2 tertiary cardio-oncology centers, illustrating the diagnostic pathway, therapeutic strategies, and clinical outcomes. Multimodality imaging was essential for initial evaluation, but histopathologic confirmation was required in all cases. Surgical biopsy proved to be a safe and effective method for tissue diagnosis. Systemic chemotherapy resulted in significant tumor regression or complete resolution in most patients, whereas surgical debulking was reserved for those with hemodynamic compromise. This case series highlights key diagnostic and management considerations that may assist clinicians facing suspected PCL.

ABSTRACT Cardiac sarcoidosis can be life‐threatening, and lack of response to immunosuppressive therapy remains a significant challenge in active disease. We present a case of refractory cardiac sarcoidosis despite third‐line immunosuppression (infliximab). Ongoing multimodality assessment is essential for managing refractory cardiac sarcoidosis.

BACKGROUND:Cardiac Resynchronisation Therapy (CRT) has demonstrated short and long-term benefit in heart failure with reduced ejection fraction (HFrEF), including ischaemic (ICM) and non-ischaemic cardiomyopathy. However, there is a paucity of evidence regarding its role in cardiac sarcoidosis (CS). METHODS:Consecutive CS patients with CRT and baseline left ventricle ejection fraction (LVEF)≤40 referred to one specialist hospital in London between November 2008-March 2023 were retrospectively reviewed. The baseline characteristics, short-term echocardiographic, clinical parameters and long-term primary and secondary outcomes were compared against a cohort of ICM patients with CRT and baseline LVEF≤40. Patients with incomplete follow-up were excluded. The primary endpoint was a composite of all-cause mortality, cardiac transplantation or heart failure hospitalisation. Secondary endpoint included ventricular arrhythmic events. RESULTS:63 CS and 93 ICM patients were analysed. A greater proportion of ICM patients male with older ages overall (both p < 0.01), whereas a larger proportion of CS patients had atrioventricular block and heart failure hospitalisations (both p < 0.01). Both cohorts demonstrated significant serial increase in left ventricular (LV) ejection fraction and reduction in LV end-systolic and end-diastolic volumes (p < 0.01). After a mean follow up of 40.9 (±32.0) months, the primary and secondary endpoint was reached by significantly more CS patients (log-rank p = 0.008 and log-rank p = 0.004). Age (HR: 1.12 (95 %CI 1.06-1.17, p < 0.001) and presence of CS (HR: 8.33 (95 %CI 3.03-22.93, p < 0.001) were independent predictors of the primary endpoint on multivariable analysis. CONCLUSION:CS patients with CRT demonstrated reverse remodelling, but had adverse long-term primary and secondary outcomes when compared to ICM patients.

18F-FDG PET with CT is an important advanced imaging modality used to assess patients with suspected or known cardiac sarcoidosis (CS). 18F-FDG PET is indicated for CS work-up in patients with extra-CS and abnormal screening results for cardiac involvement, patients under 60 y old presenting with unexplained high-grade atrioventricular heart block, and patients with suspected CS and idiopathic ventricular arrhythmias. In patients with established CS, serial 18F-FDG PET can be used to assess response to immunosuppressive therapy and long-term surveillance for reactivation of myocardial inflammation in patients with low-grade or quiescent disease. Patient preparation before 18F-FDG PET scanning is key in ensuring adequate suppression of physiologic myocardial 18F-FDG uptake, to maximize the power of the test to detect pathology. Inadequate dietary preparation can cause diffuse or focal-on-diffuse 18F-FDG uptake in the absence of active inflammation. It is important to assess resting myocardial perfusion, typically with 82Rb cardiac PET. Several different patterns of abnormalities have been reported in patients with CS, including normal myocardial perfusion with focal or patchy 18F-FDG uptake suggesting myocardial inflammation without scarring; the presence of a myocardial perfusion defect with abnormal 18F-FDG uptake suggesting myocardial scarring with inflammation; and the presence of a myocardial perfusion defect without 18F-FDG uptake indicating myocardial scarring without inflammation. Prognostically, the presence of myocardial perfusion defects and abnormal 18F-FDG uptake has been shown to be an independent predictor of death or ventricular arrythmias. A high myocardial SUVmax in the left and right ventricles has been shown to be an independent predictor of adverse clinical outcomes. Although the diagnostic performance of 18F-FDG PET has been studied, the reference standard for CS tended to rely on clinical criteria, which may be less sensitive than 18F-FDG PET at detecting CS. Therefore, the diagnosis of CS should rely on a multidisciplinary team approach involving multimodality advanced imaging, including echocardiography, cardiovascular MR, and 18F-FDG PET.

BACKGROUND:Cardiac sarcoidosis (CS) affects between 5 % to 25 % of systemic sarcoid cases. CS patients may present with heart failure (HF), with ultimate progression to advanced heart failure (AHF) associated with heightened mortality. OBJECTIVES:American guidelines emphasise using the 'I NEED HELP' criteria to identify AHF patients. The European Society of Cardiology (ESC) have alternative AHF diagnostic criteria. Both have demonstrated prognostic value, but their utility in prognosticating CS is unknown. This study aimed to address this. METHODS:109 patients, with baseline left ventricular ejection fraction (LVEF) <50 %, referred to the Royal Brompton Hospital between 2006 and 2019, were analysed. 48 patients had ≥1 'I NEED HELP' criteria consistent with AHF. Comparisons were made between the AHF and non-AHF CS patients. Sub-analysis was performed between the AHF patients that did or did not meet ESC-AHF criteria. Primary combined outcome measure was all-cause-mortality, urgent orthotopic cardiac transplant or urgent left ventricular assist device insertion. Secondary outcome measure was sustained ventricular tachycardia. RESULTS:The AHF cohort had significantly lower LVEF and higher brain natriuretic peptide values. More AHF CS patients reached combined primary outcome measure (AHF 16/48 [33 %]), than the non-AHF group (8/61 [13 %]), p < 0.019. The AHF group had shorter time to both primary and secondary events on Kaplan-Meier analysis (logrank p < 0.014 and p < 0.040 respectively). Sub-analysis revealed the ESC-AHF group had the poorest prognoses according to both outcome measures. CONCLUSIONS:In this study, both the AHA and ESC-AHF criteria had prognostic value. The ESC-AHF criteria best identifies CS patients with poorest prognoses.

Abstract Background Right ventricular (RV) involvement in cardiac sarcoidosis (CS) is associated with adverse cardiovascular outcomes. Echocardiography is recommended in the screening of sarcoidosis patients for cardiac involvement. Abnormal imaging should prompt onward performance of cardiac magnetic resonance (CMR) and 18fluorodeoxy glucose positron emission tomography (FDG-PET). Suspicion of RV involvement in the screening phase could prompt more expedient advanced imaging. Purpose This study aimed to determine how well RV-focused echocardiographic parameters could predict RV involvement of CS. We defined RV involvement by the presence of RV impairment (RVEF <50%) and/or RV late gadolinium enhancement (LGE) on CMR and/or RV FDG-uptake on FDG-PET. Methods Consecutive patients referred for evaluation of suspected CS were prospectively recruited. All those with evidence of extracardiac sarcoidosis were included. Detailed 2D and 3D echocardiography with RV strain, CMR and FDG-PET was performed in all patients within 3 months. CS was diagnosed in line with the 2014 Heart Rhythm Society criteria. RV/pulmonary artery (PA) coupling was defined as the tricuspid annular plane systolic excursion (TAPSE):pulmonary artery systolic pressure (PASP) ratio. RV 4-chamber strain included the RV free wall and septal longitudinal strain components. Results Among the 198 sarcoidosis patients enrolled, 97 (49%) were diagnosed with CS. The prevalence of pulmonary hypertension was 3%. RV involvement was present in 26 (13%) among which 14 had RV free wall or septal LGE, 17 had CMR RVEF <50% and 10 had RV FDG uptake. Six patients had both LGE and FDG uptake while 8 patients had both LGE and RVEF <50%. Compared to those without, patients with RV impairment had lower RV fractional area change (38% [IQR 28-46] vs 43% [39-48], p=0.005), RV free wall strain (-17.7 ± 4.2% vs -22.5 ± 5.5%, p<0.001), RV 4-chamber strain (-13.9 ± 3.4% vs -18.5 ± 4.5%, p<0.001), 3DE RVEF (44 ± 7.4% vs 50 ± 7.5%, p=0.019) and greater RV basal diameter (3.99 ± 0.60cm vs 3.63 ± 0.58cm, p=0.004). There was no significant difference in TAPSE (p=0.091), PASP (p=0.811), RV/PA coupling (p=0.271) and tissue doppler RV S’ (p=0.203) values between the two groups. RV 4-chamber strain (AUC: 0.797) had the best ability to predict RV involvement followed by RV free wall strain (AUC: 0.757) and 3DE RVEF (AUC: 0.730). The optimal cut-off for RV 4-chamber strain to predict RV involvement was -15.3% which had 74% sensitivity and 77% specificity. Conclusion In a prospective cohort, RV strain is a useful indicator of RV involvement among suspected CS patients and should be performed routinely in all screening studies of sarcoidosis patients.

BACKGROUND:Risk assessment in cardiac sarcoidosis remains challenging. OBJECTIVES:This study explored the prognostic value of myocardial late gadolinium enhancement (LGE) in sarcoidosis patients. METHODS:The study cohort included 324 patients with biopsy-proven sarcoidosis. LGE extent, pattern, and location were analyzed. The primary endpoint was ventricular tachycardia (VT) or ventricular fibrillation (VF) or appropriate device therapy. Secondary endpoints were hospitalization for heart failure (HF) or heart transplantation (HTx) and all-cause mortality. RESULTS:Over a 4.6-year follow-up, 30 patients (9.3%) reached the primary endpoint. HF/HTx occurred in 15 patients (4.6%) and all-cause mortality in 41 (12.7%). LGE extent was independently predictive of the primary endpoint (per SD change: HR: 1.03 [95% CI: 1.00-1.06]; P = 0.047), but not of HF/HTx (P = 0.30) or all-cause mortality (P = 0.50). Further to LGE extent, LGE on the right ventricular (RV) septum (HR: 5.43 [95% CI: 2.61-11.30]; P < 0.001), RV free wall (HR: 4.30 [95% CI: 1.99-9.27]; P < 0.001), and multifocal LGE (HR: 4.62 [95% CI: 2.19-9.72]; P < 0.001) were strongly predictive of the arrhythmia endpoint. Based on these findings, we propose an algorithm that identifies 4 patient subgroups and stratifies well the arrhythmia risk in biopsy-proven sarcoidosis patients (cumulative event rates: 1%, 11%, 23%, and 44%, respectively; chi-square = 44.7; P = 1.084 × 10-9). Compared with the Heart Rhythm Society classification system, this approach significantly enhanced model performance (chi-square = 8.02; P = 0.046) and risk discrimination (ΔAUC = 0.082; P = 0.019), and reclassified 43% of the population (9% to higher and 34% to lower risk categories). CONCLUSIONS:The authors propose a new risk stratification approach based on LGE features for assessing the risk of life-threatening ventricular arrhythmias in patients with biopsy-proven sarcoidosis.

BackgroundEchocardiography, cardiac magnetic resonance and cardiac18fluorodeoxyglucose positron emission tomography (FDG-PET) imaging play key roles in the diagnosis and management of cardiac sarcoidosis (CS), but the relative value of each modality in predicting outcomes has yet to be determined. This study sought to determine the prognostic importance of multimodality imaging data over and above demographic characteristics and left ventricular ejection fraction (LVEF).MethodsConsecutive patients newly diagnosed with CS were included. Parameters evaluated included echocardiographic regional wall motion abnormality (RWMA), myocardial strain, LVEF, right ventricular ejection fraction (RVEF), late gadolinium enhancement (LGE) extent, SUVmax and RV FDG uptake. The primary endpoint was a composite of all-cause mortality and serious ventricular arrhythmia.ResultsThe study population consisted of 208 patients with mean age of 55±13 years and LVEF of 55±12%. During a median follow-up period of 46 (IQR: 18–55) months, 14 patients died and 28 suffered serious ventricular arrhythmias. On multivariable analysis, RWMA (HR for RWMA presence 2.55, 95% CI 1.27 to 5.28, p=0.008), LGE extent (HR per 1% increase 1.02, 95% CI 1.00 to 1.04, p=0.018), RVEF (HR per 1% decrease 0.97, 95% CI 0.94 to 0.99, p=0.008) and RV FDG uptake (HR for RV FDG presence 2.48, 95% CI 1.15 to 5.33, p=0.020) were independent predictors of the primary endpoint, while LVEF was not predictive. The risk of adverse events was significantly greater in those with LGE extent ≥15% (HR for ≥15% presence 3.96, 95% CI 2.17 to 7.23, p<0.001).ConclusionIn our CS population, RWMA, LGE extent, RVEF and RV FDG uptake were strong independent predictors of an adverse outcome. These findings offer an important insight into the key multimodality imaging parameters that may be used in a future risk stratification model of patients with CS.

AbstractAimsIn cardiac sarcoidosis (CS) patients, the benefit of cardiac resynchronization therapy (CRT) remains unclear. We sought to assess the short‐term and long‐term effects of CRT in CS patients with a range of left ventricular (LV) ejection fractions (LVEFs).MethodsConsecutive CS patients with heart failure with reduced ejection fraction (HFrEF; LVEF ≤ 40%), mildly reduced ejection fraction (HFmrEF; LVEF 41%–49%) and preserved ejection fraction (HFpEF; LVEF ≥ 50) treated with CRT under the care of a tertiary UK centre between 2008 and 2023 were reviewed. CRT response was defined by >5% improvement in serial LVEF. The primary endpoint was a composite of all‐cause mortality, cardiac transplantation or unplanned hospitalization for decompensated heart failure. The secondary endpoint included ventricular arrhythmic events.ResultsOf the 100 patients enrolled (age 58 ± 10 years; 71% male), 63 had HFrEF, 17 had HFmrEF and 20 had HFpEF. After short‐term follow‐up (9.8 ± 5.4 months), HFrEF patients demonstrated significant LVEF response (P < 0.01). On Kaplan–Meier analysis (follow‐up 38 ± 32 months), HFrEF non‐responders had significantly worse event‐free survival compared with HFrEF responders for the primary (P < 0.001) and secondary (P = 0.001) endpoints. Despite short‐term LV function improvement, CRT responders still had worse event‐free survival compared with HFmrEF/HFpEF patients for the primary endpoint (P < 0.001). On multivariable Cox analysis, age [hazard ratio (HR) 1.05, 95% confidence interval (CI) 1.01–1.10, P = 0.008] and HFrEF CRT non‐response (HR 12.33, 95% CI 2.45–61.87, P = 0.002) were associated with the primary endpoint.ConclusionsIn CS patients with HFrEF, CRT response is associated with a better long‐term prognosis than non‐response. However, HFrEF CRT responders still have worse long‐term prognosis than HFmrEF/HFpEF patients.

BACKGROUND:Cardiac sarcoidosis (CS) is frequently associated with conduction abnormalities and arrhythmias. In this study, we aim to evaluate racial disparities in the frequency of arrhythmias, and associated co-morbidities, among patients with CS.METHODS:White and African American (AA) patients diagnosed with CS were identified and compared from the 2016-2020 National Inpatient Sample (NIS) database whilst adjusting for confounders via logistic regression models.RESULTS:A total of 7,935 patients with CS were included in the study. The propensity-matched sample comprised of 5,570 patients, of whom 2,785 were White and 2,785 were AA. AA patients had a longer mean length of hospital stay (LOS) (7.84 vs. 6.94, p<0.01), a higher mean Charlson Comorbidity Index (CCI) score (3.10 vs. 2.84, p<0.01), and significantly higher incidences of cardiogenic shock [(9.2% vs 6.3%, p<0.01), aOR 1.45 (95% CI 1.17-1.78), p<0.01] and acute kidney injury (AKI) [(34.3% vs. 26.9%, p<0.01), aOR 1.41 (95% CI 1.24-1.61), p<0.01]. From an arrhythmia perspective, AA CS patients were shown to have a lower frequency of: (1) ventricular tachycardia (32.5% vs. 37.9%, p<0.01), (2) ventricular fibrillation (5.4% vs.7.2%, p<0.01), (3) first-degree AV block (1.8% vs. 4.1%, p<0.01), (4) complete AV block (6.3% vs. 14.2%, p<0.01), and (5) atrial fibrillation (31.8% vs. 34.8%, p=0.016) when compared to Whites with CS. Mortality remained higher for AAs (3.8% vs. 2.7%, p=0.024).CONCLUSION:Our study demonstrates a higher incidence of cardiac arrhythmias among White patients but a higher incidence of cardiogenic shock, AKI, mean LOS, and mortality among AA patients with cardiac sarcoidosis.

BackgroundCardiac 18F-fluorodeoxyglucose (FDG)-PET-CT plays an important role for the assessment of cardiovascular diseases. Effective management of urgent scan findings facilitates optimal patient care.MethodsWe characterised the management of urgent expected and unexpected findings in patients referred for cardiac FDG-PET-CT at the Royal Brompton Hospital (UK). Urgent findings are escalated by the reporting physicians/radiologists raising RadAlert notifications to the referring clinician. We characterised the indications and time to management (TTM) between the RadAlert and the resulting management. As controls, we characterised the TTM of 33 urgent findings identified before the RadAlert system was implemented.ResultsOf the 1497 consecutive FDG-PET-CT scans screened (April 2021 to February 2023), 93 RadAlerts were suitable for analysis (TTM 7days [interquartile range: 2-14]). Expected urgent findings included active cardiac sarcoidosis (56%; TTM 8days [5-18]), heart transplant rejection (12%; 6±4days), infective endocarditis (9%; 2days [1-12]), cardiac device infections (5%; 1day [0-2]), acute myocarditis (2%; 5 and 14 days) and epicardial mass (1%; 1day). TTM did not differ significantly between indications (p=0.06). RadAlert cases had significantly shorter TTM than controls without RadAlert, p=0.001. After the RadAlerts, 81% of patients had clinical reviews and 55% had escalation of medical/surgical therapies. Unexpected findings (total n=45; median TTM 6 days [1-10]) included malignancies (n=3), infections (n=2), pneumothorax (n=1), benign diagnosis (n=30), unclear diagnosis (n=5) and 4 findings disappeared on repeat imaging.ConclusionsCardiac FDG-PET-CT identifies expected and unexpected findings in a range of cardiovascular diseases. Serious unexpected findings are rare and can be effectively escalated by the RadAlert system.