Imaging In Cardiac Sarcoidosis Research Articles

Hybrid Magnetic Resonance Positron Emission Tomography Is Associated With Cardiac-Related Outcomes in Cardiac Sarcoidosis

BackgroundImaging with late gadolinium enhancement (LGE) magnetic resonance (MR) and 18F-fluorodeoxyglucose (18F-FDG) PET allows complementary assessment of myocardial injury and disease activity and has shown promise for improved characterization of active cardiac sarcoidosis (CS) based on the combined positive imaging outcome, MR(+)PET(+). ObjectivesThis study aims to evaluate qualitative and quantitative assessments of hybrid MR/PET imaging in CS and to evaluate its association with cardiac-related outcomes. MethodsA total of 148 patients with suspected CS underwent hybrid MR/PET imaging. Patients were classified based on the presence/absence of LGE (MR+/MR−), presence/absence of 18F-FDG (PET+/PET−), and pattern of 18F-FDG uptake (focal/diffuse) into the following categories: MR(+)PET(+)FOCAL, MR(+)PET(+)DIFFUSE, MR(+)PET(−), MR(−)PET(+)FOCAL, MR(−)PET(+)DIFFUSE, MR(−)PET(−). Further analysis classified MR positivity based on %LGE exceeding 5.7% as MR(+/−)5.7%. Quantitative values of standard uptake value, target-to-background ratio, target-to-normal-myocardium ratio (TNMRmax), and T2 were measured. The primary clinical endpoint was met by the occurrence of cardiac arrest, ventricular tachycardia, or secondary prevention implantable cardioverter-defibrillator (ICD) before the end of the study. The secondary endpoint was met by any of the primary endpoint criteria plus heart failure or heart block. MR/PET imaging results were compared between those meeting or not meeting the clinical endpoints. ResultsPatients designated MR(+)5.7%PET(+)FOCAL had increased odds of meeting the primary clinical endpoint compared to those with all other imaging classifications (unadjusted OR: 9.2 [95% CI: 3.0-28.7]; P = 0.0001), which was higher than the odds based on MR or PET alone. TNMRmax achieved an area under the receiver-operating characteristic curve of 0.90 for separating MR(+)PET(+)FOCAL from non-MR(+)PET(+)FOCAL, and 0.77 for separating those reaching the clinical endpoint from those not reaching the clinical endpoint. ConclusionsHybrid MR/PET image–based classification of CS was statistically associated with clinical outcomes in CS. TNMRmax had modest sensitivity and specificity for quantifying the imaging-based classification MR(+)PET(+)FOCAL and was associated with outcomes. Use of combined MR and PET image–based classification may have use in prognostication and treatment management in CS.

Abstract 13286: Co-Morbidities Clustering According to Imaging Diagnostic Criteria in Cardiac Sarcoidosis

Background: Cardiac sarcoidosis (CS) diagnostic guidelines updated in 2006 and in 2017 now include imaging findings of left ventricular (LV) dysfunction (LV EF < 50%) on cardiac MRI and echocardiography as major diagnostic criterion. Limited data is available with regards to co-morbidities clustering in sarcoidosis and CS according to the 2017 updated guideline diagnostic criteria. Methods: A case control, single tertiary medical center study included 558 sarcoidosis patients with documented extracardiac sarcoidosis and completed electrocardiogram and/or cardiac MRI imaging. CS 2006 and 2017 diagnostic criteria and co-morbidity data were extracted from electronic charts and were available for comparison in 540 patients. Results: The total study population was composed of 52.7% (281/540) females, aged 58.9+/-12.6 years old, with diabetes mellitus (DM) present in 29.6% (160/540), HTN in 51.7% (279/540), CAD in 13.3% (72/540), moderate to severe aortic, mitral, or tricuspid valvular disease in 14.8% (80/540), ESRD in 2.4% (13/540), and COPD or asthma in 21.9% (118/540). There was a significant clustering of co-morbidities according to the imaging CS diagnostic criteria. Patients meeting the 2017 CS imaging criteria were found to be older (61.4+/-11.3 vs 58.8+/-12.8 years old in patients not meeting criteria, p=0.085), predominantly male (69.2%, (54/78) vs. 44.4%, (205/462), p<0.001), and demonstrated significantly increased prevalence of DM (39.7%, (31/78) vs. 27.9%, (129/462), p=0.034), and valvular disease (33.3%, (26/78) vs. 11.7%, (54/462), respectively, p<0.001). However, there were no statistically significant difference in the prevalence of HTN (60.3%, (47/78) vs. 50.2%, (232/462), p=0.101), ESRD (3.9%, (3/76) vs. 2.2%, (10/456), p=0.361), and COPD or asthma (19.2%, (15/78) vs. 22.3%, (103/462), p=0.544) in patients meeting 2017 CS criteria compared to those that did not. Conclusion: CS patients meeting 2017 imaging diagnostic criteria were found to have statistically significant clustering of the following co-morbidities: male gender, DM, and valvular disease. These findings underscore importance of comprehensive care in CS patients and warrant caution in assigning LV abnormalities observed on imaging solely to CS etiology.

Imaging in Cardiac Sarcoidosis: Complementary Role of Cardiac Magnetic Resonance and Cardiac Positron Emission Tomography

Imaging in Cardiac Sarcoidosis: Complementary Role of Cardiac Magnetic Resonance and Cardiac Positron Emission Tomography

Current State and Future Directions of Multimodality Imaging in Cardiac Sarcoidosis.

Cardiac sarcoidosis (CS) is an increasingly recognized cause of heart failure and arrhythmia. Historically challenging to identify, particularly in the absence of extracardiac sarcoidosis, diagnosis of CS has improved with advancements in cardiac imaging. Recognition as well as management may require interpretation of multiple imaging modalities. Echocardiography may serve as an initial screening study for cardiac involvement in patients with systemic sarcoidosis. Cardiac magnetic resonance imaging (CMR) provides information on diagnosis as well as risk stratification, particularly for ventricular arrhythmia in the setting of late gadolinium enhancement. More recently, 18F-fluorodeoxyglucose position emission tomography (FDG-PET) has assumed a valuable role in the diagnosis and longitudinal management of patients with CS, allowing for the assessment of response to treatment. Hybrid FDG-PET/CT may also be used in the evaluation of extracardiac inflammation, permitting the identification of biopsy sites for diagnostic confirmation. Herein we examine the approach to diagnosis and management of CS using multimodality imaging via a case-based review.

PET Imaging in Cardiac Sarcoidosis: A Narrative Review with Focus on Novel PET Tracers.

Sarcoidosis is a multi-system inflammatory disease characterized by the development of inflammation and noncaseating granulomas that can involve nearly every organ system, with a predilection for the pulmonary system. Cardiac involvement of sarcoidosis (CS) occurs in up to 70% of cases, and accounts for a significant share of sarcoid-related mortality. The clinical presentation of CS can range from absence of symptoms to conduction abnormalities, heart failure, arrhythmias, valvular disease, and sudden cardiac death. Given the significant morbidity and mortality associated with CS, timely diagnosis is important. Traditional imaging modalities and histologic evaluation by endomyocardial biopsy often provide a low diagnostic yield. Cardiac positron emission tomography (PET) has emerged as a leading advanced imaging modality for the diagnosis and management of CS. This review article will summarize several aspects of the current use of PET in CS, including indications for use, patient preparation, image acquisition and interpretation, diagnostic and prognostic performance, and evaluation of treatment response. Additionally, this review will discuss novel PET radiotracers currently under study or of potential interest in CS.

The Role of Multimodality Imaging in Cardiac Sarcoidosis.

The etiology and the progression of sarcoidosis remain unknown. However, cardiac sarcoidosis (CS) is significantly associated with a poor prognosis due to the associated congestive heart failure, arrhythmias (such as an advanced atrioventricular block), and ventricular tachyarrhythmia. Novel imaging modalities are now available to detect CS lesions secondary to active inflammation, granuloma formation, and fibrotic changes. 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) and cardiac magnetic resonance imaging (CMR) play essential roles in diagnosing and monitoring patients with confirmed or suspected CS. The following focused review will highlight the emerging role of non-invasive cardiac imaging techniques, including FDG PET/CT and CMR.

The role of right ventricular late gadolinium enhancement scoring by cardiac magnetic resonance imaging in cardiac sarcoidosis

Abstract Background The right ventricular (RV) function is a significant predictor of the prognosis and exercises tolerance in patients with left ventricular dysfunction. The previous study reported that RV late gadolinium enhancements (LGE) as assessed by cardiac magnetic resonance (CMR) imaging were present in 16% of pulmonary sarcoidosis. Although RV LGE is associated with a poor prognosis, a semi-quantitative assessment of RV LGE was not been established so far. Moreover, the frequency of RV dysfunction (RVD) in cardiac sarcoidosis (CS) and predictors of RVD are unclear. Purpose Our aim of this study is to establish RV LGE semi-quantitative scores and to clarify the rate and predictors of RVD in CS. Methods Forty-four consecutive patients with CS who underwent CMR imaging (median age, 63 [IQR 54–71] years old; 10 males) were studied. They were diagnosed as definitive based on the Heart Rhythm Society expert consensus statement. Two patients were excluded due to no long axial view. Thus, a total of 42 patients were included in this study. RV LGE was semi-quantitatively evaluated using the original score developed by ours. RV was classified into RV outflow tract (OT), basal, middle and apex. RVOT and apex are assigned 1 segment for each, and basal and middle are assigned 4 segments include anterior, septal, inferior and lateral (Figure1). In total, 10 segments were evaluated. RV LGE scores were analysed independently by the two operators. The RV ejection fraction was assessed in a long axial view without an RVOT (20 phases, 12 slices). We divided the cohort into the non-RVD group (RVEF ≥45%) and the RVD group (RVEF <45%) and compared the RV scores between the two groups. Results The RVD was present in 31% of the entire cohort. The presence of RV LGE was significantly higher in the RVD group than the non-RVD group (83% vs. 43%, p=0.037). RV LGE score in the RVD group was significantly higher than that in the non-RVD group (2.83±1.95 vs. 0.83±1.21, p=0.001). The septal lesions in basal and middle levels accounted for 54% in all segments. RVEF showed a significant inverse correlation with RV LGE score (r=−0.57, p<0.0001). In the ROC curve for RVD, the RV LGE score of more than 2 and equal could discriminate RVD from non-RVD with AUC of 0.813 (Sensitivity = 0.77, Specificity = 0.76, p=0.003). Conclusion RVD was observed in 31% of patients with definitive CS. The septal lesions were the most frequent in the RV LGE site, which suggests that the longitudinal abnormal motion of RV is a significant predictor of the RVD in patients with CS. RV LGE score is a valuable method to assess RVD. RV LGE score Funding Acknowledgement Type of funding source: None

Diagnostic Accuracy of Advanced Imaging in Cardiac Sarcoidosis

Background The diagnostic yield of cardiac sarcoidosis (CS) by endomyocardial biopsy is limited. Fluorodeoxyglucose (FDG) positron emission tomography (PET) and cardiac magnetic resonance imaging (MRI) may facilitate noninvasive diagnosis, but the accuracy of this approach is not well defined. We aimed to correlate findings from FDG PET and cardiac MRI with histological findings from explanted hearts of patients who underwent cardiac transplantation. Methods We analyzed the explanted heart histology for all patients who underwent cardiac transplant at our center from April 2008 to July 2018 and had pretransplant FDG PET (n=18) or cardiac MRI (n=31). The likelihood of CS based on FDG PET or cardiac MRI was categorized in a blinded fashion using a previously published method. RESULTS: Using a CS probable cutoff for FDG PET resulted in a sensitivity of 100.0% (95% CI, 54.1%-100.0%) and a specificity of 33.3% (95% CI, 9.9%-65.1%). Three of the 9 CS probable by FDG PET cases were found to be arrhythmogenic cardiomyopathy. The test characteristics of cardiac MRI are more challenging to comment on using our data as there was only one confirmed case of CS on post-transplant histological assessment. Of the 8 CS highly probable or probable cases by cardiac MRI, 3 were found to be dilated cardiomyopathy, and 2 were found to be end-stage hypertrophic cardiomyopathy. Conclusions FDG PET and cardiac MRI can help facilitate the diagnosis of CS in patients with advanced heart failure with a high degree of sensitivity but lower specificity.

Electrophysiology study for risk stratification in patients with cardiac sarcoidosis and abnormal cardiac imaging

BackgroundAbnormalities on cardiac imaging (cardiac magnetic resonance imaging [CMR] or positron emission tomography [PET]), left ventricular ejection fraction (LVEF), and electrophysiology study (EPS) all predict ventricular arrhythmias (VA) in patients with cardiac sarcoidosis (CS). We sought to assess the utility of EPS in patients with CS and abnormal cardiac imaging, focusing on those with LVEF >35%. MethodsWe identified all patients treated at our institution from 2000 to 2017 who: 1.) had probable or definite CS; 2.) had either late gadolinium enhancement (LGE) on CMR or abnormal 18-flourodeoxyglucose (FDG) uptake on PET, and 3.) had undergone EPS. The primary endpoint was VA during follow up. ResultsTwenty five patients were included, of whom 10 (40%) had positive EPS. During a mean follow-up of 4.8 +/− 3.4 years, 11 (44%) patients had VA. The positive predictive value (PPV) of EPS for VA was 100% and the negative predictive value (NPV) of EPS for VA was 93%. Among 12 patients with LVEF >35% and no prior VA, the PPV of EPS for VA was 100% and the NPV of EPS for VA was 90%. ConclusionEPS may help with risk stratification in patients with CS and abnormal imaging, especially those without conventional indications for ICD placement. Among patients with LVEF >35% and no history of prior VA, a negative EPS has good positive and negative predictive value for future VA events.

Updates on the Role of Imaging in Cardiac Sarcoidosis.

The non-specific symptom profile and subclinical nature of disease along with variable region of cardiac involvement in systemic sarcoidosis make the diagnosis particularly challenging. The yield of endomyocardial biopsy, a gold standard for diagnosis, is not high unless coupled with additional imaging modalities to detect regional involvement. This review is focused on highlighting the major recent advances in imaging modalities and diagnosis of cardiac sarcoidosis. There has been much interest and increasing research focused on developing newer and improved imaging modalities to establish diagnosis. CMR and 18F- FDG-PET are now considered imaging modalities of choice in most centers worldwide, but the data comparing both methodologies head-to-head is limited. Nevertheless, novel radiotracers (i.e. 68Ga-DOTANOC, 18F-Flurpiridaz, 13N-Ammonia) and hybrid combination PET/CMR imaging are coming to spotlight with improved sensitivity and specificity for earlier detection of myocardial sarcoid. As CMR and PET are showing increased utilization in cardiac sarcoidosis, 201Th-SPECT, 99mTc MDP SPECT, 67Ga Scintigraphy, and 82Rb PET are falling out of favor. Newer imaging modalities, radionuclide tracers, and hybrid PET/CMR combinations have been promising in better detecting cardiac sarcoidosis and are currently being evaluated in larger trials.

A joint procedural position statement on imaging in cardiac sarcoidosis: from the Cardiovascular and Inflammation & Infection Committees of the European Association of Nuclear Medicine, the European Association of Cardiovascular Imaging, and the American Society of Nuclear Cardiology.

This joint position paper illustrates the role and the correct use of echocardiography, radionuclide imaging with 18F-fluorodeoxyglucose positron emission tomography, radionuclide myocardial perfusion imaging and cardiovascular magnetic resonance imaging for the evaluation and management of patients with known or suspected cardiac sarcoidosis. This position paper will aid in standardizing imaging for cardiac sarcoidosis and may facilitate clinical trials and pooling of multi-centre data on cardiac sarcoidosis. Proposed flow charts for the work up and management of cardiac sarcoidosis are included.

Multimodality Imaging in Cardiac Sarcoidosis: Is There a Winner?

Sarcoidosis is a multisystem granulomatous disease of unknown cause that can affect the heart. Cardiac sarcoidosis may be present in as many as 25% of patients with systemic sarcoidosis, and it is frequently underdiagnosed. The early and accurate diagnosis of myocardial involvement is challenging. Advanced imaging techniques play important roles in the diagnosis and management of patients with cardiac sarcoidosis.

Focus Issue on Cardiac Sarcoidosis from ICNC-12 Symposium on Advanced Imaging in Cardiac Sarcoidosis

Focus Issue on Cardiac Sarcoidosis from ICNC-12 Symposium on Advanced Imaging in Cardiac Sarcoidosis

Tissue Doppler imaging in cardiac sarcoidosis

A middle-aged African lady, who presented with ventricular tachycardias, mitral valve regurgitation and congestive heart failure, was diagnosed with cardiac sarcoidosis. Tissue Doppler imaging demonstrated abnormalities suggestive of myocardial scar, which was confirmed by contrast-enhanced cardiac magnetic resonance.

Thallium-201 and gallium 67 single photon emission computed tomographic imaging in cardiac sarcoidosis

Thallium-201 and gallium 67 single photon emission computed tomographic imaging in cardiac sarcoidosis