Cardiac Sarcoidosis Research Articles

Isolated Cardiac Sarcoidosis Masquerading as Peripheral Vertigo: A Case Report

Isolated Cardiac Sarcoidosis Masquerading as Peripheral Vertigo: A Case Report

Quantitative cardiac magnetic resonance standardized signal intensity comparison in dilated cardiomyopathy vs. cardiac sarcoidosis.

Dilated cardiomyopathy (DCM) and cardiac sarcoidosis (CS) manifest unique late gadolinium enhancement (LGE) patterns on cardiac magnetic resonance (CMR), indicative of different myocardial scar distributions. However, the overlap in these patterns due to their lack of specificity complicates differentiation. This study introduces a novel quantitative method employing z-score analysis of LGE-CMR intensity to objectively compare the spatial distribution of LGE intensity between DCM and CS. This retrospective study included 22 NICM patients (13 DCM, 9 CS) who underwent CMR before electrophysiology study from November 2018 to May 2023. LGE images were delineated into sub-endocardial, mid-myocardial, and sub-epicardial layers across anterior, lateral, inferior, and septal walls using the AHA 17-segment model. CMR signal intensities were standardized to z-scores (z = (x - μ)/σ), with x as the signal intensity for a specific myocardial segment, and μ and σ as the mean and SD for all LV myocardial segments, to map regional intensity variations. Compared to DCM, CS patients exhibited significantly higher CMR signal intensity z-scores in the septum (β = 0.32, p = 0.009), particularly in the endocardial third of the right ventricular (RV) side (β = 0.56, p = 0.001). A z-score greater than 0.40 in this area was associated with a CS diagnosis, with an area under the ROC curve of 0.692 in fivefold cross-validation. Patients with CS exhibit higher affinity for contrast in the septum, particularly on the RV endocardium. Standardized analysis of CMR signal intensities provides a novel, quantitative method for distinguishing CS from DCM, with the former exhibiting higher CMR signal intensity z-scores in the septum.

Assessing prognostic outcomes in cardiac sarcoidosis with advanced heart failure: How do current guidelines fare?

Assessing prognostic outcomes in cardiac sarcoidosis with advanced heart failure: How do current guidelines fare?

Cardiac Sarcoidosis Presenting with High-Grade Atrioventricular Block: The Importance of Multimodality Imaging.

Cardiac Sarcoidosis Presenting with High-Grade Atrioventricular Block: The Importance of Multimodality Imaging.

Changes in Rhythm: A Fatal Case of Progressive Cardiac Sarcoidosis Presenting With Recurrent and Refractory Ascites

Changes in Rhythm: A Fatal Case of Progressive Cardiac Sarcoidosis Presenting With Recurrent and Refractory Ascites

Predictors of Poor Long-Term Outcomes in Patients with Newly Diagnosed Asymptomatic Cardiac Sarcoidosis: A Cardiovascular Magnetic Resonance Study

Background: The prevalence of patients with cardiac sarcoidosis (CS) diagnosed at a subclinical stage has increased; however, their long-term outcomes are not well known. Objectives: To investigate the incidence and predictors of adverse long-term outcomes in newly diagnosed patients with asymptomatic CS. Methods: Forty-three patients with newly diagnosed asymptomatic CS and comprehensive baseline evaluation with cardiovascular magnetic resonance (CMR) were studied. Asymptomatic CS was defined as CS in patients with biopsy-proven extracardiac sarcoidosis without cardiac symptoms but with abnormalities on CMR or positron emission tomography according to Heart Rhythm Society criteria. The primary endpoint was a composite of all-cause mortality, new ventricular arrhythmia or an atrioventricular block requiring cardiac device implantation, and hospitalization for heart failure. Results: Patients had a mean age of 56 ± 11 years and presented with normal left ventricular (LV) ejection fraction (58 ± 4%). A total of 44.2% of patients reached the composite endpoint during 5 years of follow-up. Patients with the primary endpoint were predominantly female (73.7%) and had a significantly higher prevalence of right ventricular (RV) involvement compared to patients without the primary endpoint (RV late gadolinium enhancement (LGE) in 26.3% vs. 4.2%, p = 0.037). In multivariate regression analysis, extensive LV LGE (HR 1.61, 95% CI 1.16–2.04, p = 0.004) and impaired RV global longitudinal strain (GLS) at baseline (HR 0.46, 95% CI 0.24–0.68, p = 0.015) were significantly predictive of the primary endpoint, whereas treatment with corticosteroids after CS diagnosis was significantly associated with improved outcomes (HR 7.69, 95% CI 1.11–11.11, p = 0.044). Conclusions: Newly diagnosed patients with asymptomatic CS have a significant incidence of adverse outcomes after 5 years of follow-up. The extent of LV LGE and impaired RV GLS at baseline predict poor long-term outcomes in asymptomatic CS.

Differentiating Cardiac Sarcoidosis from Arrhythmogenic Right Ventricular Cardiomyopathy: A Systematic Review.

Cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) are distinct disorders with different pathophysiologic pathways, but they share similar clinical presentations that could lead to misdiagnosis and inappropriate therapeutic decisions. We searched PubMed and Google Scholar databases and other relevant literature to retrieve comparative studies including CS and ARVC that were published before 2024. The National Heart, Lung and Blood Institute checklist was used for quality assessment and the review was conducted according to the PRISMA guidelines. Three reviewers determined study eligibility and made quality assessments. A total of seven studies were included in the review. Patients with CS were older (five of seven studies) and had more comorbidities (two of two studies). PR interval (four of five studies) and QRS duration (four of four studies) were longer in CS. Most studies reported lower left ventricular ejection fraction in CS (five of six studies), and septal involvement on cardiac MRI was more common in CS (two of three studies). 18-Fluorodeoxyglucose uptake on positron emission tomography (PET) scan was seen in up to 90% of CS patients. 62.5% to 100% of patients with CS fulfilled 1994 or 2010 International Task Force criteria for ARVC. Available evidence suggest that atrioventricular and intraventricular conduction defects in an older (>40 years) patient with low left ventricular ejection fraction should raise suspicion for CS, especially when other supportive findings such as 18-fluorodeoxyglucose avidity on PET were present. Neither 1994 nor 2010 ARVC Task Force criteria should be used to discriminate CS from ARVC.

18F-FDG PET/CT diagnosis of cardiac sarcoidosis with third-degree atrioventricular block: two cases report

18F-FDG PET/CT diagnosis of cardiac sarcoidosis with third-degree atrioventricular block: two cases report

Early Corticosteroid Therapy and Its Effect on Ventricular Tachyarrhythmia Recurrence in Cardiac Sarcoidosis: A 10-Year Longitudinal Multicenter Study.

The role of corticosteroids in the management of ventricular tachyarrhythmia (VA) in cardiac sarcoidosis (CS) remains controversial. This study assessed the effect of early steroid initiation on VA recurrence rates in patients with CS and evaluated VA frequency before and after steroid therapy. From a registry of 137 patients with CS with arrhythmias, those with VA and more than 60 days of follow-up were included. Patients were categorized into three groups: previous-on-steroid, those using corticosteroids at the time of the first VA (n = 15); early-steroid, those with steroid initiation within 60 days (n = 21); and no-or-delayed-steroid, those without steroid use at 60 days from the first VA (n = 35). The VA recurrence and timing were analyzed. The median age at the first VA was 60 years. Over a median follow-up period of 81 (range: 4-345) months, 43 patients experienced VA recurrence. The median time to recurrence was 101, 33, and 17 months in the early-steroid, no-or-delayed steroid, and previous-on-steroid groups, respectively. Corticosteroid use at 60 days was an independent predictor of VA recurrence at 120 months (hazard ratio: 2.257; p = 0.045 in the no-or-delayed steroid group and hazard ratio: 3.155; p = 0.021 in the previous-on-steroid group vs. the early-steroid group). Low left ventricular ejection fraction (LVEF) was associated with frequent VA episodes. Corticosteroid initiation did not increase early VA episodes. Early corticosteroid initiation after VA onset reduced the long-term recurrence without increasing early episodes. Cases of repetitive VA were common in those with low LVEF.

Management of patients with myocarditis and arrhythmogenic phenotype.

Acute myocarditis (AM) is an inflammatory condition of the myocardium that may lead to severe complications, including acute heart failure and life-threatening ventricular arrhythmias (VAs). In-hospital VAs are estimated to affect 2.5% of adult patients with AM. Recent insights suggest a genetic predisposition to develop VA in a subset of patients with AM. This review will focus on arrhythmogenic manifestations of AM, highlighting risk stratification for VA after an acute episode and the contribution of genetic factors, emphasizing the need to integrate clinical, imaging, and genetic findings. In addition, prognostic information derived from cardiac magnetic resonance imaging will be discussed, pointing out the association between VA and the presence, extension, and septal localization of late gadolinium enhancement. The overlap between inherited arrhythmogenic and inflammatory cardiomyopathies will be explored, with specific attention to the identification of desmosomal gene variants, which are associated with recurrent myocarditis-like episodes and a higher risk of VA. Cardiac sarcoidosis, giant cell myocarditis, and immune checkpoint inhibitors-related myocarditis will be discussed as a paradigm of inflammatory cardiomyopathies with increased arrhythmic burden. Finally, the clinical challenges of managing patients with AM and arrhythmogenic presentation will be tackled, looking at indications for implantable cardioverter defibrillators after the acute phase.

Relation between resting amygdala activity and cardiovascular events in patients with cardiac sarcoidosis.

Previous studies indicate amygdala activity (AmygA) measured by FDG-positron emission tomography (PET) predicts the risk of subsequent cardiovascular events. However, AmygA measurement use for predicting the prognosis of patients with cardiac sarcoidosis (CS) is unknown. We aimed to investigate the association between AmygA and cardiovascular events in patients with CS. Consecutive 40 patients with CS who underwent FDG-PET were identified retrospectively. Cardiovascular events included ventricular tachyarrhythmias and heart failure hospitalizations. We examined the association between AmygA and cardiovascular events and investigated the timing of FDG-PET influence on AmygA measurements. During a follow-up of 4.5 (2.5-7.7) years, 14 (35%) patients experienced cardiovascular events. Left-AmygA was a stronger cardiovascular event predictor than the Right-AmygA, and incidences were significantly higher in the high left-AmygA group than low group (log-rank P = 0.018). Multivariable analysis revealed left-AmygA augmentation (hazard ratio [HR]: 1.76 per 0.1 increase, 95% confidence interval [CI]: 1.12-2.87, P = 0.016) was the only independent predictor of cardiovascular event. Among the 40 patients, 32 underwent multiple PET-scans. No significant difference was found between the AmygA value on the first PET-scan and the mean on multiple PET scans, and the correlation coefficient was 0.93 (P < 0.001). Multivariate analyses revealed the mean left-AmygA value was the only independent predictor of cardiovascular event (P = 0.011). High left-AmygA was associated with the higher rates of cardiovascular events in patients with CS. AmygA would be a prognostic biomarker regardless of the PET-scan timing.

Statistical modeling to determine severity of respiratory sarcoidosis and parameters associated with cardiac sarcoidosis: as a way to stratify the risk of developing pulmonary hypertension

Statistical modeling to determine severity of respiratory sarcoidosis and parameters associated with cardiac sarcoidosis: as a way to stratify the risk of developing pulmonary hypertension

Cardiac Magnetic Resonance Imaging in the Evaluation and Prognosis of Infiltrative Cardiomyopathies.

Advancements in cardiac magnetic resonance (CMR) imaging quality and availability have made it an essential tool in the care of individuals living with cardiomyopathies. CMR complements clinical suspicion, electrocardiogram patterns, and echocardiographic findings to help elucidate the etiology of cardiomyopathies and can also be used to prognosticate and follow treatment responses. In this review, we highlight the common CMR findings in cardiac amyloidosis, cardiac sarcoidosis, iron overload cardiomyopathy, and Fabry disease. We also summarize prognostic findings and additional potential roles for CMR in the management of infiltrative cardiomyopathies.

Prevalence of ventricular parasystole in patients with cardiac sarcoidosis: correlation between parasystole and inflammation in ventricular fibrillation

BackgroundVentricular parasystole is strongly associated with ventricular fibrillation (VF) in patients with non-ischaemic cardiomyopathy. However, the relationship between ventricular parasystole and cardiac sarcoidosis (CS) remains unclear. The purpose of this...

Durable remission of cardiac sarcoidosis following discontinuation of methotrexate: A prospective cohort study.

Durable remission of cardiac sarcoidosis following discontinuation of methotrexate: A prospective cohort study.

Differential response of myocardium and His bundle to steroid therapy in a patient with cardiac sarcoidosis: A change of pacing threshold in His bundle pacing with cardiac sarcoidosis.

A patient with cardiac sarcoidosis who had introduced His bundle pacing and caused threshold deterioration was treated with steroid therapy, which resulted in lead threshold improvement and avoidance of lead replacement. Additionally, the thresholds of improvement with steroid therapy vary between the myocardium and His bundle in these patients.

Long term prognosis in cardiac sarcoidosis under FDG-PET guided immunosuppressive therapy.

Long term prognosis in cardiac sarcoidosis under FDG-PET guided immunosuppressive therapy.

Atrial FDG uptake and atrial fibrillation: A systematic review and meta-analysis.

Atrial FDG uptake and atrial fibrillation: A systematic review and meta-analysis.

Advanced Imaging in the Diagnosis of Cardiac Sarcoid and LV Noncompaction.

Advanced Imaging in the Diagnosis of Cardiac Sarcoid and LV Noncompaction.

Characteristics and outcomes of hospitalized patients with Isolated and systemic cardiac sarcoidosis: Analysis of the Nationwide readmissions database 2016-2021.

Characteristics and outcomes of hospitalized patients with Isolated and systemic cardiac sarcoidosis: Analysis of the Nationwide readmissions database 2016-2021.