Myocardial Inflammation: Focus on Cardiac Sarcoidosis

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.

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MYOCARDIAL INFLAMMATION/FIBROSIS DETECTION BY PULSE-CANCELLATION ECHOCARDIOGRAPHY IN PATIENTS WITH CARDIAC SARCOIDOSIS-CORRELATION WITH FDG-PET

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(19)32101-1 ◽

2019 ◽

Vol 73 (9) ◽

pp. 1495 ◽

Cited By ~ 1

Author(s):

Lane Zhang ◽

Steven Fein ◽

Ping Lu ◽

Fantauzzi John ◽

David Steckman ◽

...

Keyword(s):

Fdg Pet ◽

Cardiac Sarcoidosis ◽

Myocardial Inflammation

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Utility of updated diagnositc criteria to detect isolated cardiac sarcoidosis

European Heart Journal ◽

10.1093/ehjci/ehaa946.2146 ◽

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

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Cardiac Sarcoidosis: When and How to Treat Inflammation

Cardiac Failure Review ◽

10.15420/cfr.2021.16 ◽

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.

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HIGH RISK OF MALIGNANT VENTRICULAR ARRHYTHMIAS IN CARDIAC SARCOIDOSIS ASSOCIATED WITH FAILURE TO SUPPRESS MYOCARDIAL INFLAMMATION ASSESSED BY FDG-PET SCANNING

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(16)31824-1 ◽

2016 ◽

Vol 67 (13) ◽

pp. 1823

Author(s):

Ashley Pender ◽

Peter H.S. Sporn ◽

Susan R. Russell ◽

Rod Passman ◽

Bradley Knight ◽

...

Keyword(s):

High Risk ◽

Fdg Pet ◽

Ventricular Arrhythmias ◽

Cardiac Sarcoidosis ◽

Myocardial Inflammation ◽

Pet Scanning

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Exercise intolerance — from spiroergometry to transdiaphragmatic myocardial punch biopsy: a case report of isolated cardiac sarcoidosis

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa121 ◽

2021 ◽

Author(s):

Michael Johannes Schindler ◽

Ardan M Saguner ◽

Stefano Benussi ◽

Peter Karl Bode ◽

Robert Manka ◽

...

Keyword(s):

Cardiac Sarcoidosis ◽

Treatment Options ◽

Transoesophageal Echocardiography ◽

Punch Biopsy ◽

Exercise Intolerance ◽

Myocardial Inflammation ◽

High Dose ◽

Av Block ◽

Electrocardiographic Monitoring ◽

Caseating Granuloma

Abstract Background Several aetiologies account for exercise intolerance, with cardiac sarcoidosis (CS) constituting a rare cause thereof. The pathogenesis of CS is still unresolved and its diagnosis still difficult to establish, in the absence of any extracardiac manifestations in particular. Case summary A 49-year-old amateur athlete presented with exercise intolerance during running over a 3-week period. Coronary artery and structural lung disease were excluded by coronary angiography and computer tomography. The symptoms could be reproduced during spiroergometry during which an exercise-induced high-degree atrioventricular (AV) block was documented. During electrocardiographic monitoring, a 2:1 AV block was observed. Different imaging modalities showed inferobasal septal inflammation and fibrosis. Transthoracic and transoesophageal echocardiography-guided endomyocardial biopsies were inconclusive and only subsequent epicardial biopsy performed by transdiaphragmatic minimally invasive surgery lead to the histological diagnosis of non-caseating granuloma, confirming CS. The patient was treated with high-dose steroids 1 week after implantation of a primary prevention dual-chamber implantable cardioverter-defibrillator (ICD). While tapering steroids, recurrence of myocardial inflammation occurred. However, no tachytherapies and <0.1% right ventricular pacing were needed after 2 years of follow-up. Discussion Differential diagnoses were either an infiltrative disease, a tumour, or an infectious disease. Due to the different treatment options, we had to establish definite diagnosis by myocardial biopsy. Retrospectively, the implantation of the ICD can be discussed. However, cardiac magnetic resonance imaging showed fibrosis which is usually irreversible and substrate for potentially lethal ventricular arrhythmia. Confirming the diagnosis of isolated CS is challenging. Long-term management should be guided individually based on clinical and imaging findings.

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