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.

Gene panels represent a widely used strategy for genetic testing in a vast range of Mendelian disorders. While this approach aids reliable bioinformatic detection of short coding variants, it often fails to detect many larger variants. Recent studies have recommended the adoption of pangenome references (as opposed to linear reference genomes like GRCh38) to augment detection of large variants from targeted sequencing, potentially providing diagnostic laboratories with the possibility to streamline diagnostic work-ups and reduce costs. Here, we analyze 1969 cardiomyopathy cases and 1805 controls sequenced with the Illumina Trusight Cardio panel using a pangenome-based workflow (GRAF) and five conventional orthogonal methodologies (GATK HaplotypeCaller, GATK-gCNV, ExomeDepth, Manta and Lumpy-SV) to detect variants ≥ 20 bp in size. Following lab-based variant validation by means of PCR and Sanger sequencing, we show that GRAF conjugates higher precision and recall (F1 score 0.86) compared with other methods (F1 0-0.57) in detecting potentially pathogenic variants ≥ 20 bp from short-read panel data. Results were complemented by a comparison of the tools’ performance in detecting ground truth variants on reference sample HG002 from Genome In A Bottle, which confirmed GRAF to outperform other tools also on exome sequencing (F1 0.97 vs. 0-0.94). Notably, in the HG002 benchmark dataset, GRAF also showed slightly improved performance compared to GATK HaplotypeCaller in the identification of small variants (1–19 bp; F1 0.975 vs. 0.968). Our results indicate that pangenome-based workflows aid improved detection of large variants from targeted sequencing data in the clinical context and suggest that they may contribute to more unified variant detection frameworks for all-size genetic variants in the future.

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.

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 and Aims Microstructural disturbances underlie dysfunctional contraction and adverse left ventricular (LV) remodelling after ST-elevation myocardial infarction (STEMI). Biphasic diffusion tensor cardiovascular magnetic resonance (DT-CMR) quantifies dynamic reorientation of sheetlets (E2A) from diastole to systole during myocardial thickening, and markers of tissue integrity [mean diffusivity (MD) and fractional anisotropy (FA)]. This study investigated whether microstructural alterations identified by biphasic DT-CMR: (i) enable contrast-free detection of acute myocardial infarction (MI); (ii) associate with severity of myocardial injury and contractile dysfunction; and (iii) predict adverse LV remodelling. Methods Biphasic DT-CMR was acquired 4 days (n = 70) and 4 months (n = 66) after reperfused STEMI and in healthy volunteers (HVOLs) (n = 22). Adverse LV remodelling was defined as an increase in LV end-diastolic volume >= 20% at 4 months. MD and FA maps were compared with late gadolinium enhancement images. Results Widespread microstructural disturbances were detected post-STEMI. In the acute MI zone, diastolic E2A was raised and systolic E2A reduced, resulting in reduced E2A mobility (all P < .001 vs. adjacent and remote zones and HVOLs). Acute global E2A mobility was the only independent predictor of adverse LV remodelling (odds ratio .77; 95% confidence interval .63-.94; P = .010). MD and FA maps had excellent sensitivity and specificity (all > 90%) and interobserver agreement for detecting MI presence and location. Conclusions Biphasic DT-CMR identifies microstructural alterations in both diastole and systole after STEMI, enabling detection of MI presence and location as well as predicting adverse LV remodelling. DT-CMR has potential to provide a single contrast-free modality for MI detection and prognostication of patients after acute STEMI.

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.

Introduction: In patients with cardiac sarcoidosis (CS), implantable cardioverter-defibrillators (ICDs) are important for preventing sudden cardiac death. This study aimed to investigate sex disparities in CS patients undergoing ICD implantation. Methods: The 2016-2020 National Inpatient Sample (NIS) database compared the characteristics and outcomes of males and females with CS receiving ICDs. Results: Among 760 CS patients who underwent inpatient ICD implantation, 66.4% were male. Males were younger (55.0 vs. 56.9 years, p < .01), had higher rates of diabetes (31.7% vs. 21.6%, p < .01) and chronic kidney disease (CKD) (16.8% vs. 7.8%, p < .01) but lower prevalence of atrial fibrillation (AF) (11.9% vs. 23.5%, p < .01), sick sinus syndrome (4.0% vs. 7.8%, p = .024), ventricular fibrillation (VF) (9.9% vs. 15.7%, p = .02), and black ancestry (31.9% vs. 58.0%, p < .01). Unadjusted major adverse cardiovascular events (MACE), defined as a composite of in-hospital death, myocardial infarction (MI), and ischemic stroke, was higher in females (11.8% vs. 6.9%, p = .024), but when adjusted for age and tCharlson Comorbidity Index (CCI), females demonstrated significantly lower odds of experiencing MACE (aOR: 0.048, 95% CI: 0.006-0.395, p = .005). Incidence of acute kidney injury (AKI) post-ICD was significantly lower in females (15.7% vs. 23.8%, p = .01) as was the adjusted odds (aOR: 0.282, 95% CI: 0.146-0.546, p < .01). There was comparable mean length of stay and hospital charges. ConclusionI: CD utilization in CS patients is more common among males, who have a higher prevalence of diabetes and CKD but a lower prevalence of AF, sick sinus syndrome, and VF. Adjusted MACE and AKI were significantly lower in females.

Background: The highly arrhythmogenic nature of cardiac sarcoidosis (CS) leads to high morbidity and mortality, the rates of which may be higher in COVID-19 patients. This study aimed to evaluate the outcomes of CS patients admitted to hospitals with COVID-19. Methods: The study utilised the 2020-2021 National Inpatient Sample database, examining primary COVID-19 cases in adults aged older than or equal to 18 years. Those with CS were identified using ICD-10 code "D86.85" and compared with and without propensity matching (1: 10) to those without CS for baseline characteristics and primary outcomes of acute kidney injury (AKI), use of mechanical ventilation, cardiac arrest and mortality. Results: In total, 2 543 912 COVID-19 cases were identified. Before propensity matching, CS patients were more likely to be younger (58.0 vs. 64.0 years, P<0.01), male (64.0% vs. 52.6%, P=0.011), of Black ethnicity (60.0% vs. 15.9%, P<0.01), exhibit higher Charlson Comorbidity Index (CCI) scores (3.00 vs. 1.00, P<0.01) and had a higher incidence of in-hospital cardiac arrest (aOR 2.649, 95% CI 1.366-5.134, P=0.004). After propensity matching (CS, N=95; non-CS, N=875), those with CS were at a statistically significant reduced risk of AKI (aOR 0.484, P=0.01); however, the outcomes of death, cardiac arrest, mechanical ventilation, length of stay (LOS) and healthcare costs did not reach significance. Conclusion: In a propensity-matched cohort admitted with COVID-19, CS patients had a reduced risk of AKI, but comparable LOS, rates of cardiac arrest, mechanical ventilator use, and mortality. Future research is warranted to develop evidence-based guidelines for managing COVID-19 in patients with CS.

Cardiac sarcoidosis is associated with significant morbidity and mortality. Immunosuppressive treatment focuses on suppressing myocardial inflammation, which can lead to major adverse events especially when progressing to fibrosis. Conventional management usually includes steroids and steroid sparing agents such as methotrexate and azathioprine. Tumour necrosis factor alpha inhibitors are often reserved for those with a worsening clinical status and/or evidence of persistent inflammatory activity despite conventional therapy. Refractory cardiac sarcoidosis (CS) can be defined as the persistence or progression of active disease, evidenced either by lack of clinical response or persistence or progression of imaging abnormalities, despite being on conventional therapy. In the United Kingdom, tumour necrosis factor alpha inhibitors are currently not licensed for cardiac sarcoidosis as there are no randomised controlled trials to assess the efficacy of infliximab in this patient cohort. In this study, we present the outcomes of six patients treated with infliximab for refractory cardiac sarcoidosis at Royal Brompton Hospital and performed a systematic review of the existing literature on use of infliximab in cardiac sarcoidosis. We searched the Cochrane Library, OVID Medline, OVID Embase, Web of Science and Pubmed to identify 7 full-text studies assessing the role of infliximab in the management of cardiac sarcoidosis. Infliximab was found to play a vital role in stabilising refractory cardiac sarcoidosis by stemming clinical deterioration, arrythmia burden and even reducing steroids requirements. Further prospective trial data is necessary to validate these findings.

Background: There is conflicting evidence regarding the response to a fixed dose of regadenoson in patients with high body weight. The aim of this study was to evaluate the effectiveness of regadenoson in patients with varying body weights using novel quantitative cardiovascular magnetic resonance (CMR) perfusion parameters in addition to standard clinical markers. Methods: Consecutive patients with typical angina and/or risk factors for coronary artery disease (N = 217) underwent regadenoson stress CMR perfusion imaging using a dual-sequence quantitative protocol with perfusion parameters generated from an artificial intelligence (AI)-based algorithm. CMR was performed on 1.5T scanners using a standard 0.4 mg injection of regadenoson. A cohort of consecutive patients undergoing adenosine stress perfusion (N = 218) was used as a control group. Results: An inverse association of myocardial perfusion reserve and weight (mean decrease -0.05 per 10 kg increase, 95% confidence interval [CI] -0.009/-0.0001, P = 0.045) was noted in the regadenoson group but not in patients stressed with adenosine (P = 0.77). Adjusted logistic regression analysis revealed a 10 kg increase resulted in 36% increased odds for inadequate stress response (odds ratio [OR] = 1.36, 95% CI 1.10-1.69, P = 0.005). Moreover, a significant interaction (OR = 1.09, 95% CI 1.02-1.16, P = 0.012) between stressor type (regadenoson vs adenosine) and weight was noted. This was also confirmed in the propensity-matched subgroup (P = 0.024) and was not attenuated after adjustment (P = 0.041). Body surface area (BSA) (P = 0.006) but not body mass index (P = 0.055) was differentially associated with inadequate response conditional to the stressor used, and this association remained significant after adjustment for confounders (P = 0.025). Patients in the highest quartile of weight (> 93 kg) or BSA (> 2.06 m2) had substantially increased odds for inadequate response with regadenoson (OR = 8.19, 95% CI 2.04-32.97, P = 0.003 for increased weight and OR = 7.75, 95% CI 1.93-31.13, P = 0.004 for increased BSA). Both weight and BSA had excellent discriminative ability for inadequate regadenoson response (receiver operating characteristic area under curves 0.84 and 0.83, respectively). Conclusion: Using quantitative perfusion CMR in patients undergoing pharmacological stress with regadenoson, we found an inverse relationship between patient weight and both clinical response and myocardial perfusion parameters. A fixed-dose bolus approach may not be adequate to induce maximal hyperemia in patients with increased weight. Weight-adjusted stressors, such as adenosine, may be considered instead in patients with body weight > 93 kg and BSA > 2.06 m2.

IntroductionSarcoidosis is a systemic inflammatory disorder characterised by non-caseating granulomas. Cardiac sarcoidosis (CS) normally causes conduction abnormalities, ventricular arrhythmias, and heart failure. Little is known about the characteristics and impact of sarcoidosis in patients admitted with ST-elevation myocardial infarction (STEMI). This study aims to fill this void.Material and methodsUtilising the National Inpatient Sample (NIS) database (2016–2020), individuals with STEMI were identified and categorised based on sarcoidosis presence whilst adjusting for confounders via logistic regression models.ResultsAmong 851,290 STEMI patients, 1215 had sarcoidosis. Before propensity matching, sarcoidosis patients were notably different in demographics and comorbidities compared to non-sarcoidosis patients. After propensity score matching (PSM), sarcoidosis patients were found to have a higher incidence of supraventricular tachycardia (SVT) (2.5% vs. 1.3%, p = 0.024) and acute kidney injury (AKI) (23.3% vs. 20.8%, aOR = 1.269, 95% CI: 1.02–1.58, p = 0.033) but a lower incidence of undergoing coronary artery bypass graft (CABG) (5.5% vs. 8.5%, aOR = 0.663; 95% CI: 0.472–0.931, p = 0.018), while no significant disparities were noted in PCI, cardiogenic shock, mortality, or mean length of stay (LOS).ConclusionsUsing propensity-matched large real-world data of STEMI patients, sarcoidosis was associated with fewer cases of CABG and a greater incidence of AKI and SVT compared to non-sarcoidosis patients.

BACKGROUND:Cardiac sarcoidosis (CS) is an inflammatory condition that can present with heart failure (HF). Cardiac resynchronization therapy (CRT) is known to improve clinical outcomes for patients with left bundle branch block in the general HF population. However, data about the outcomes of CRT in CS is limited. METHODS:A systematic literature search was conducted using PubMed/Medline, Embase, and the Cochrane Library from inception to February 2024 to identify studies that reported clinical outcomes following the use of CRT in patients with CS. Data for outcomes was extracted, pooled, and analyzed. OpenMetaAnalyst was used for pooling untransformed proportions along with the corresponding 95 % confidence intervals (CIs). RESULTS:Five studies with a total of 176 CS patients who received CRT were included. The pooled incidence for all-cause mortality was 7.6 % (95 % CI: -3 % to 18 %), for HF-related hospitalizations 23.2 % (95 % CI: 2 % to 43 %), and for major adverse cerebral and cardiovascular events 27 % (95 % CI: 8 % to 45 %) after a mean follow-up of 60.1 (±48.7) months. The pooled left ventricular ejection fraction (LVEF) was 34.28 % (95 % CI: 29.88 % to 38.68 %) demonstrating an improvement of 3.75 % in LVEF from baseline LVEF of 30.58 % (95 % CI: 24.68 % to 36.48 %). The mean New York Heart Association (NYHA) functional class was 2.16 (95 % CI: 1.47 to 2.84) after CRT as compared to the baseline mean NYHA of 2.58 (95 % CI: 2.29 to 2.86). CONCLUSION:Although improvements were observed in LVEF and mean NYHA, mortality was high in CS patients with CRT.

BackgroundThere is a paucity of evidence on impact of a delay in Cardiac Sarcoidosis (CS) diagnosis after high-grade atrioventricular-block (AVB) and this study aims to fill this void.MethodsConsecutive CS patients (n=77) with high grade AVB referred to one specialist hospital in London between February 2007 to February 2023 were retrospectively reviewed. The median time from AVB to diagnosing CS (112 days) was used to define the Early (n=38) and Late (n=39) cohorts. The primary endpoint was a composite of all-cause mortality, cardiac transplantation, ventricular arrhythmic events or heart failure hospitalisation. Secondary endpoints included difference in maintenance prednisolone dose, need for cardiac device upgrade and device complications.ResultsThe mean age of the cohort was 54.4 (±10.6) years of whom 64% were male and 81% Caucasian. After a mean follow up of 54.9 (±45.3) months, the primary endpoint was reached by more patients from the Late cohort (16/39 vs 6/38, p=0.02; multivariable HR 6.9; 95%CI 1.5-32.2, p=0.01). Early Group were more likely to have received an Implantable Cardioverter Defibrillator or Cardiac Resynchronisation Therapy-defibrillator as index device after AVB (19/38 vs 6/39; p<0.01) and had fewer device upgrades (19/38 vs 30/39, p=0.01) and a trend towards fewer device complications (1 vs 5, p=0.20). The maintenance dose of prednisolone was significantly higher in Late Group [20.7(±9.7) mg vs 15.3(±7.9) mg, p=0.02].ConclusionA late diagnosis of CS was associated with more adverse events, a greater probability of needing a device upgrade and required higher maintenance steroid dose.