scholarly journals THE RELATIONSHIP BETWEEN MYOCARDIAL INFLAMMATION, FIBROSIS AND PACING BURDEN IN CARDIAC SARCOIDOSIS

2021 ◽  
Vol 77 (18) ◽  
pp. 318
Author(s):  
Amtul Mansoor ◽  
David Chang ◽  
James Gabriels ◽  
Josephine Rini ◽  
Kenneth Nichols ◽  
...  
Keyword(s):  
Cardiac Sarcoidosis ◽  
Myocardial Inflammation ◽  
The Relationship

scholarly journals Quantitative analysis of myocardial metabolic heterogeneity is superior to visual assessment for the detection of active cardiac sarcoidosis by F-18 FDG PET-CT imaging

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Malik ◽  
M Yazdani ◽  
SM Gould ◽  
E Reyes
Keyword(s):  
Fdg Pet ◽  
Cardiac Sarcoidosis ◽  
Visual Assessment ◽  
Ct Imaging ◽  
Myocardial Inflammation ◽  
Fdg Uptake ◽  
Pet Ct ◽  
Metabolic Heterogeneity ◽  
Fdg Pet Ct

Abstract Funding Acknowledgements Type of funding sources: None. Background Myocardial inflammation may occur in the context of a multisystem disease such as sarcoidosis, adversely affecting prognosis. A definitive diagnosis of cardiac sarcoidosis (CS) is essential to implementing life-saving treatment but this is complicated by the invasive nature of endomyocardial biopsy (EMB) and its low accuracy. Positron emission tomography (PET) assists in diagnosis, which relies on visual interpretation of myocardial F-18 FDG uptake. The value of quantitative analysis and its application to clinical practice remain uncertain. Purpose To investigate the power of quantitative F-18 FDG PET-CT imaging analysis for detecting CS in patients with suspected disease. Methods All patients underwent F-18 FDG PET-CT after a 24-hour low-carbohydrate diet and 15-hour fasting as part of their diagnostic work-up for suspected cardiac inflammation. Cardiovascular magnetic resonance acted as gatekeeper to PET-CT in 8 of every 10 scans. Myocardial F-18 FDG uptake was assessed qualitatively and quantitatively using both manually drawn regions of interest and automatic polar maps to measure global and segmental standardised F-18 FDG uptake values (SUV).  The coefficient of variation (CoV) was calculated to determine uptake heterogeneity. To confirm diagnosis, follow-up data regarding disease progression, further testing and treatment were collected. To allow for sufficient follow-up time, the first 40 consecutive patients from a prospective registry (n= 214; Sep 2017-Jun 2020) were included. Results A comprehensive clinical picture was obtained successfully in 37 patients (median [IQR], 17 [13.5] months) and a final diagnosis of CS reached in 7 (disease prevalence, 19%). EMB was performed in 2 patients only while 3 underwent PPM/ICD implantation. Significant predictors of CS were fulfilment of Japanese Ministry of Health and Welfare criteria (Wald, 6.44; p = 0.01) and left ventricular dysfunction (Wald 6.72; p = 0.01). Qualitative F-18 FDG PET-CT had a high negative (95%) but low positive (45%) predictive value for CS (sensitivity, 83%; specificity, 77%). F-18 FDG SUV CoV was the strongest imaging predictor (Wald, 6.77; p = 0.009) and was significantly higher in CS than non-CS (CoV median [quartiles], 0.26 [0.21, 0.36] and 0.12 [0.11, 0.14] respectively; p = 0.004). As per ROC curve analysis (AUC, 0.84), a CoV threshold of 0.20 was highly specific (93%) and sensitive (86%) for CS. Conclusion In a referring population with a low prevalence of cardiac sarcoidosis, F-18 FDG PET-CT imaging is sensitive for the detection of myocardial inflammation with active disease unlikely in patients with a negative scan. Quantitative evaluation of metabolic heterogeneity within the myocardium provides a strong, independent marker of active disease and should be considered alongside visual assessment.

scholarly journals Myocardial Inflammation, Sports Practice, and Sudden Cardiac Death: 2021 Update

Medicina ◽  
2021 ◽  
Vol 57 (3) ◽  
pp. 277
Author(s):  
Paolo Compagnucci ◽  
Giovanni Volpato ◽  
Umberto Falanga ◽  
Laura Cipolletta ◽  
Manuel Antonio Conti ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cardiac Magnetic Resonance ◽  
Sudden Cardiac Death ◽  
Magnetic Resonance ◽  
Cardiac Death ◽  
Current Knowledge ◽  
Myocardial Inflammation ◽  
Resonance Imaging ◽  
Inflammatory Cardiomyopathy ◽  
The Relationship

Myocardial inflammation is an important cause of cardiovascular morbidity and sudden cardiac death in athletes. The relationship between sports practice and myocardial inflammation is complex, and recent data from studies concerning cardiac magnetic resonance imaging and endomyocardial biopsy have substantially added to our understanding of the challenges encountered in the comprehensive care of athletes with myocarditis or inflammatory cardiomyopathy (ICM). In this review, we provide an overview of the current knowledge on the epidemiology, pathophysiology, diagnosis, and treatment of myocarditis, ICM, and myopericarditis/perimyocarditis in athletes, with a special emphasis on arrhythmias, patient-tailored therapies, and sports eligibility issues.

scholarly journals Utility of updated diagnositc criteria to detect isolated cardiac sarcoidosis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Sato ◽  
M Yamamoto ◽  
T Ishizu ◽  
M Ieda
Keyword(s):  
Diagnostic Criteria ◽  
Clinical Diagnosis ◽  
Fdg Pet ◽  
Cardiac Involvement ◽  
Cardiac Sarcoidosis ◽  
Histological Finding ◽  
Left Ventricular ◽  
Myocardial Inflammation ◽  
Histologic Diagnosis ◽  
Anti Inflammatory

Abstract Background Prior study reported around one-third of cardiac sarcoidosis (CS) are considered as isolated CS. Detection of CS is challenging due to the limited sensitivity of endomyocardial biopsy and applicability of guidelines, especially in patients without extra-cardiac involvement. Existing diagnostic criteria by Japanese Ministry of health and Welfare (JMHW) or Heart Rhythm Society (HRS) require the presence of extra-cardiac sarcoidosis for clinical diagnosis, isolated CS is not diagnosable in the absence of a positive histological finding. Recently, Japanese Society of Cardiology (JCS) updated diagnostic criteria for CS, which provides the pathway to diagnose isolated CS. Purpose We aimed to assess the reliability of the updated CS guideline in diagnosing CS compared to the prior guidelines. Methods We retrospectively identified 162 consecutive patients who underwent FDG-PET for suspected CS from 2012 through 2019. According to the updated JCS diagnostic criteria, patients were classified as histologic diagnosis of CS, clinical diagnosis of CS, or isolated CS (Figure A). We compared the association between diagnostic criteria and response with anti-inflammatory therapy. Results The JCS criteria classified 24 patients (15%) as having clinical CS, 4 (3%) as histological diagnosis of CS, and 21 (13%) as isolated CS. The JMHW criteria defined 22 patients (14%) as having CS (clinical 11%, histological 3%) and HRS criteria classified 11 patients (7%) as having CS (clinical 4%, histological 3%). Extra-cardiac involvement was detected in 36 patients (22%) with 8% of histological confirmation. Among the 126 patients without extra-cardiac involvement, prevalence of cardiac involvement was higher in isolated CS (P<0.05 for all). Compared with clinical diagnosis group, patients with isolated CS showed higher incident of regional wall motion abnormality (WMA) or left ventricular (LV) dysfunction (p=0.023). In the subgroup of 45 patients with serial FDG-PET evaluation, only updated CS criteria was associate with improvement in myocardial inflammation by FDG-PET (p<0.001). Conclusions Updated JCS diagnostic criteria detects CS patients with active myocardial inflammation which require anti-inflammatory therapy regardless of extra-cardiac involvement better than the prior guidelines. Diagnostic criteria for CS Funding Acknowledgement Type of funding source: None

Myocardial Inflammation: Focus on Cardiac Sarcoidosis

2021 ◽  
pp. 165-187
Author(s):  
David J. Murphy ◽  
Sharmila Dorbala
Keyword(s):  
Cardiac Sarcoidosis ◽  
Myocardial Inflammation

scholarly journals Cardiac Sarcoidosis: When and How to Treat Inflammation

2021 ◽  
Vol 7 ◽  
Author(s):  
Gerard T Giblin ◽  
Laura Murphy ◽  
Garrick C Stewart ◽  
Akshay S Desai ◽  
Marcelo F Di Carli ◽  
...  
Keyword(s):  
Cardiac Involvement ◽  
Cardiac Sarcoidosis ◽  
Multidisciplinary Treatment ◽  
Current Evidence ◽  
Myocardial Inflammation ◽  
Evidence Based ◽  
Future Directions ◽  
Evidence Based Treatment ◽  
Inflammatory Phase ◽  
Treatment Indications

Sarcoidosis is a complex, multisystem inflammatory disease with a heterogeneous clinical spectrum. Approximately 25% of patients with systemic sarcoidosis will have cardiac involvement that portends a poorer outcome. The diagnosis, particularly of isolated cardiac sarcoidosis, can be challenging. A paucity of randomised data exist on who, when and how to treat myocardial inflammation in cardiac sarcoidosis. Despite this, corticosteroids continue to be the mainstay of therapy for the inflammatory phase, with an evolving role for steroid-sparing and biological agents. This review explores the immunopathogenesis of inflammation in sarcoidosis, current evidence-based treatment indications and commonly used immunosuppression agents. It explores a multidisciplinary treatment and monitoring approach to myocardial inflammation and outlines current gaps in our understanding of this condition, emerging research and future directions in this field.

scholarly journals Ventricular tachycardia in cardiac sarcoidosis -prognosis, characterization of ventricular substrates and outcomes of treatment-

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
R Tonegawa ◽  
K Miyamoto ◽  
N Ueda ◽  
K Nakajima ◽  
M Wada ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Cardiac Sarcoidosis ◽  
Tertiary Care ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Pharmacologic Therapy ◽  
Ventricular Ejection Fraction ◽  
Fdg Uptake ◽  
The Relationship

Abstract Background The prognosis, the underlying substrate and clinical outcomes of treatment are unclear in patients with cardiac sarcoidosis (CS)-related ventricular tachycardia (VT). Objective This study investigated the prognosis and the relationship between electroanatomical mapping (EAM) and imaging findings in patients with CS-related VT. Methods A total of 203 CS patients (Age 68.1±11.6 years, 87 males) were enrolled at two tertiary care medical centers between 2000 and 2018. All met the 2016 Japanese Circulation Society guidelines for diagnosis of CS. They were followed for a composite of major adverse cardiac events (MACE) including cardiac death, heart transplantation, unscheduled hospitalization for heart failure, and life-threatening ventricular arrhythmias. Distribution of late gadolinium enhancement (LGE) on cardiac MRI (CMR) and/or an abnormal myocardial 18F-fluorodeoxyglucose (FDG) uptake on positron emission tomography at diagnosis were examined. The relationship between EAM and the image findings were also analyzed in patients with radiofrequency ablation (RFA) for VT. Results During a median follow-up of 53 months, 87 of the 203 patients (43%) experienced a MACE. Baseline factors associated with MACE were presence of sustained VT (HR, 2.43, 95% CI 1.54–3.85, P<0.001), left ventricular ejection fraction below 50% (HR, 1.95 95% CI 1.07–3.56, P=0.029), and abnormal myocardial FDG uptake (HR, 2.42 95% CI 1.04–5.61, P=0.039). Overall, 69 of the 203 patients (34%) experienced sustained VT. Abnormal myocardial FDG uptake was significantly more prevalent in patients with VT than in those without (92.7% vs. 78.5%, P=0.02). A total of 25 patients (9.9%) required RFA for CS-related VT (Age 64.0±8.7 years, 12 males, 1.32±0.56 RFAs per patient). Abnormal electrocardiograms (EGM) were observed in 22 of the 25 patients (88%). LGE was more frequent than abnormal FDG uptake in areas with an abnormal EGM (77% vs. 41%; P=0.002). Over a mean follow-up period of 67-months, 13 of the 25 patients with RFA (52%) remained free of VT episodes (Figure). VT recurred in nine of the 12 patients with RFA and in 17 of the 47 patients without RFA, but was suppressed by intensive pharmacologic therapy such as the combined use of amiodarone and sotalol. In patients with CS-related VT, survival without experiencing a MACE did not differ in participants with or without RFA. Conclusions In our 203 CS patients, sustained VT and abnormal FDG uptake were associated with worse cardiac outcomes. The prevalence of abnormal FDG uptake was significantly higher in patients with CS-related VT, LGE on CMR was more frequent within localized areas of an abnormal EGM, suggesting that both scar itself and the associated inflammation were involved in the pathogenesis of CS-related VT. Successful RFA of CS-related VT is still challenging, and recurrence is common. Preprocedural CMR can be useful in detecting abnormal EGMs that are potential targets for substrate ablation. Funding Acknowledgement Type of funding source: None

Cardiac function at diagnosis is important prognostic factor in patients with cardiac sarcoidosis -from Japanese nationwide questionnaire survey-

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Kamada ◽  
K Ishibashi ◽  
K Nakajima ◽  
N Ueda ◽  
T Kamakura ◽  
...  
Keyword(s):  
Prognostic Factor ◽  
Adverse Events ◽  
Cardiac Function ◽  
Corticosteroid Therapy ◽  
Questionnaire Survey ◽  
Cardiac Sarcoidosis ◽  
Cardiovascular Death ◽  
Cardiac Events ◽  
Relationship Of ◽  
The Relationship

Abstract Background Sarcoidosis is a systemic non-caseating granulomatous disease of unknown etiology. Cardiac involvement (cardiac sarcoidosis, CS) has been reported to be an important prognostic factor in this disease because of heart failure and/or ventricular arrhythmia, and corticosteroid therapy is usually prescribed to prevent cardiac events. However, little is known about the relationship of cardiac function and concomitant corticosteroid therapy on later cardiac events in CS. Objective We evaluated the relationship between prognosis and left ventricular ejection fraction (LVEF) at the time of diagnosis in CS patients from the Japanese nationwide questionnaire survey. Methods Total of 757 Japanese patients from 57 hospitals who diagnosed CS were examined. Patients who unsatisfied the criteria of the Japanese new guidelines, or who underwent cardiac transplantations were excluded, and 420 patients (287 females, mean age 60±13 years old, median follow-up periods 1864 days [interquartile range: 845–3159 days]) were analyzed. The relationship of adverse events (all-cause death, cardiovascular death, and appropriate ICD [Implantable Cardioverter Defibrillator] discharge) and LVEF (with corticosteroid 84%) (low LVEF: LVEF≤35% n=98 [with corticosteroid in 78%], moderate LVEF: LVEF 35–50% n=104 [with corticosteroid in 93%], normal LVEF: 50≤LVEF n=218 [with corticosteroid in 83%]) were evaluated respectively. Results 89 CS patients developed all-cause death (n=50), cardiovascular death (n=30) or appropriate ICD discharge (n=48). The frequency of corticosteroid therapy was not different in the each LVEF group, but Kaplan-Meier analysis revealed that all-cause death, cardiovascular death, and all cardiovascular adverse events were more observed in lower LVEF group (log-rank p<0.0001). Furthermore, multivariate Cox hazard analysis revealed that LVEF was a most important independent prognostic factor in CS. Conclusion This Japanese nationwide questionnaire survey data showed that initial LVEF was an independent and strong prognostic predictor in CS, therefore primary prevention would be needed even after starting corticosteroid in patients with decreased cardiac function. Funding Acknowledgement Type of funding source: None

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