The prevalence of cardiac sarcoidosis is underestimated: [18F]FDG-PET is the answer

Abstract Funding Acknowledgements Type of funding sources: None. Background Overt cardiac involvement is reported in 5% of patients with sarcoidosis, although autopsy and imaging studies suggest higher prevalence, worldwide variation. The role of 18F-fluorodeoxyglucose positron emission tomography ([18F]FDG-PET) in non-invasive diagnosis and follow-up has increased in the last decade. Purpose Our goal is to describe the prevalence, clinical manifestations and outcomes of cardiac sarcoidosis (CS), diagnosed through [18F]FDG-PET, in a southern European population. Methods We included all patients with histological diagnosis of extracardiac sarcoidosis screened with [18F]FDG-PET between 2009 and 2020. We collected data on clinical manifestations, cardiac magnetic resonance (CMR) results, and mortality outcomes and compared those with and without cardiac involvement. We applied the criteria for the diagnosis of CS from Heart Rhythm Society. Results Of the 400 patients screened with [18F]FDG-PET, 128 had a histological diagnosis of extracardiac sarcoidosis (54.7% females, mean age 51.0 ± 14.2 years). None underwent endomyocardial biopsy. Ten patients had a pattern of [18F]FDG uptake consistent with CS defined as diffuse (n = 5), focal (n = 3), and focal on diffuse (n = 2). Of the 128 patients, 14 also underwent CMR, which identified 2 subjects with positive findings in both modalities and 3 additional patients: focal (n = 1), multifocal mid-wall (n = 2), focal mid-wall (n = 2), and multifocal subepicardial (n = 1) delayed gadolinium enhancement. Overall, 13 patients (10.2%) fulfilled the criteria for probable CS (53.8% female, mean age 56.2 ± 12.6 years), all with multiorgan involvement, mostly lung and lymph nodes (each 92%), followed by skin and central nervous system (each 15%). Median left ventricle ejection fraction was 62% [55-65] and there were cardiac manifestations of CS in 6 patients (46%): sick sinus syndrome (n = 2), complete heart block (n = 1), frequent premature ventricular complexes (n = 1), ventricular tachycardia plus heart failure (n = 1), and bifascicular block plus heart failure (n = 1). Eleven patients (85%) with probable CS were medicated with immunosuppressant drugs: corticosteroids (n = 9), methotrexate (n = 4), and azathioprine (n = 2). Four patients with previous [18F]FDG screening were revaluated after treatment, each showing no cardiac uptake. After a mean follow-up of 4.0 ± 1.0 years, mortality was three-fold higher in patients with cardiac involvement, despite the absence of statistical significance (15% vs. 5%, P = 0.151). Conclusions In a southern European population with histological extracardiac sarcoidosis, the prevalence of cardiac involvement was 10.2%, most asymptomatic. [18F]FDG-PET improves the diagnostic yield and plays an important role in monitoring response to therapy. The higher mortality trend in those with CS needs to be ascertained in longer follow-up.

Download Full-text

Natural evolution of cardiac sarcoidosis in an asymptomatic patient: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytz099 ◽

2019 ◽

Vol 3 (3) ◽

Cited By ~ 2

Author(s):

Ganna Degtiarova ◽

Olivier Gheysens ◽

Johan Van Cleemput ◽

Wim Wuyts ◽

Jan Bogaert

Keyword(s):

Computed Tomography ◽

Late Gadolinium Enhancement ◽

Cardiac Involvement ◽

Cardiac Sarcoidosis ◽

Clinical Signs ◽

Steroid Treatment ◽

Lung Pathology ◽

Gadolinium Enhancement ◽

18F Fdg

Abstract Background Sarcoidosis is a multi-organ granulomatous disease of unknown aetiology. Adverse outcome related with cardiac involvement, makes early diagnosis of cardiac sarcoidosis crucial. Case summary In a 55-year-old man presenting with recurrent pulmonary infections, computed tomography (CT) showed several enlarged mediastinal lymph nodes and no lung pathology. Subsequent mediastinoscopy revealed the diagnosis of sarcoidosis. Further screening for organ involvement showed multifocal cardiac involvement both on cardiac magnetic resonance (CMR) and 18-F-fluorodeoxyglucose-positron emission tomography-computed tomography (18F-FDG PET-CT). Because of the lack of functional deterioration and clinical symptoms, no steroid treatment was initiated and regular follow-up of cardiac abnormalities was performed by CMR. Unremarkable progression of cardiac involvement during the first 2 years of follow-up turned into a dramatic involvement after 4 years, with the increase in the number and size of lesions at late gadolinium enhancement (LGE) CMR. Late gadolinium enhancement areas matched the regions of strongly increased 18F-FDG uptake. For the first time, the patient started complaining on shortness of breath, electrocardiography showed an atrioventricular block Grade 1. Cardiac biomarkers and cardiac function were still preserved. Steroid treatment was started. Although an electrophysiology study was negative, Holter monitoring showed ventricular arrhythmia. Cardioverter-defibrillator was implanted. Discussion This case shows the progression of cardiac sarcoidosis on CMR in an asymptomatic untreated patient over a 4-year period, and rises the awareness of possible severe cardiac damage even in the absence of clinical signs of cardiac involvement. Combination of PET and CMR is appealing to better understand the evolution of cardiac sarcoidosis and may help in the management of such patients.

Download Full-text

Management of Cardiac Sarcoidosis in 2020

Arrhythmia & Electrophysiology Review ◽

10.15420/aer.2020.09 ◽

2020 ◽

Vol 9 (4) ◽

pp. 182-188

Author(s):

Nisha Gilotra ◽

David Okada ◽

Apurva Sharma ◽

Jonathan Chrispin

Keyword(s):

Heart Failure ◽

Heart Block ◽

Ventricular Arrhythmias ◽

Cardiac Involvement ◽

Cardiac Sarcoidosis ◽

Management Strategies ◽

Clinical Manifestations ◽

Future Directions ◽

Inflammatory Phase

Sarcoidosis is an inflammatory granulomatous disease that can affect any organ. Up to one-quarter of patients with systemic sarcoidosis may have evidence of cardiac involvement. The clinical manifestations of cardiac sarcoidosis (CS) include heart block, atrial arrhythmias, ventricular arrhythmias and heart failure. The diagnosis of CS can be challenging given the patchy infiltration of the myocardium but, with the increased availability of advanced cardiac imaging, more cases of CS are being identified. Immunosuppression with corticosteroids remains the standard therapy for the acute inflammatory phase of CS, but there is an evolving role of steroid-sparing agents. In this article, the authors provide an update on the diagnosis of CS, including the role of imaging; review the clinical manifestations of CS, namely heart block, atrial and ventricular arrhythmias and heart failure; discuss updated management strategies, including immunosuppression, electrophysiological and heart failure therapies; and identify the current gaps in knowledge and future directions for cardiac sarcoidosis.

Download Full-text

P121918F-FDG perfusion-metabolism mismatch 3 days after acute myocardial infarction predicts worse outcome: molecular glucose steel phenomenon visualized by hybrid PET-MRI images

European Heart Journal ◽

10.1093/eurheartj/ehz748.0178 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

I J Pavo ◽

M Gyongyosi ◽

A Jakab ◽

D Lukovic ◽

K Zlabinger ◽

...

Keyword(s):

Myocardial Infarction ◽

Heart Failure ◽

Acute Myocardial Infarction ◽

Infarct Size ◽

Fdg Pet ◽

Tracer Uptake ◽

Fdg Uptake ◽

Infarcted Area ◽

18F Fdg

Abstract Background The disturbance of cardiac energy substrate metabolism has a decisive role in the pathogenesis of adverse cardiac remodeling and heart failure. Under normoxic conditions, the free fatty acid metabolism is the predominant pathway by providing the highest energy per substrate molecule. Severe myocardial hypoxia leads to a switch from beta-oxidation to glucose metabolism to increase the ATP production per oxygen molecule. This metabolic disorder appears as perfusion-metabolism mismatch in 18F-fluorodeoxyglucose (18F-FDG) PET images, as increased 18F-FDG uptake in the under-perfused hypoxic myocardial area. Purpose The aim of our study was to evaluate the simultaneous perfusion, metabolism and function of the ischemic heart by hybrid 18F-FDG-PET-cMRI with late enhancement images in a translation animal model of heart failure. Methods Under general anesthesia, closed chest reperfused acute myocardial infarction (AMI) was induced in 36 domestic pigs by 90 min occlusion of the mid left anterior descending artery with a percutaneous intracoronary balloon, followed by reperfusion. Three days and 1 month after AMI, after 12h fasting, 18F-FDG-PET-cMRI were performed by using standardized acquisition protocols (n=30). Cardiac functional parameter, such as ejection fraction (EF), end-diastolic volume (EDV), infarct size, and mean tracer uptake of the infarcted area were quantitatively assessed. Six animals were euthanized after the 3-day 18F-FDG-PET-cMRI images to elaborate the differences in gene expression patterns in animals with perfusion-metabolic mismatch by using next generation sequencing (NGS) and pathway network analyses. Results Eight (group Mismatch) of the 30 animals (group Match) with 1-month follow-up showed high 18F-FDG uptake in the infarcted area (perfusion-metabolism mismatch) at the 3-day 18F-FDG-PET-cMRI-LE images (Figure). The animals in the Mismatch group had significantly lower EF at 3 days (34±8.8 vs 42±3%) and at the 1-month follow-up (35.8±6 vs 43±6.6%) and larger infarct size at day 3 (26.6±6.6 vs 22.1±4.4%) and 1 month (28±5.4 vs 20.3±4.3%) with higher EDV at 1 month. Mean tracer uptake of the infarcted area was significantly reduced in the Mismatch group at 1 month (56±23.1 vs 64.7±13.2%). NGS revealed downregulation of the cholesterol metabolism pathway, and upregulation of carbohydrate derivative catabolism pathway with highly activated innate immune system and genes responsible for cytokine activation in the infarcted area 3 days post-AMI in the Mismatch group, which explains the paradox high 18F-FDG tracer uptake in the infarction zone. Accordingly, the high energy demand of the severe hypoxic area led to “glucose steel phenomenon” at the molecular level, subtracting the 18F-FDG from the normally perfused non-ischemic myocardial regions. Conclusions 18F-FDG-glucose perfusion-metabolism mismatch early after AMI visualized by hybrid 18F-FDG-PET-MRI images predict development of LV adverse remodeling. Acknowledgement/Funding Fibrotarget EU Grant Nr 602904

Download Full-text

[18F]FDG-PET in cardiac sarcoidosis: A single-centre study in a southern European population

International Journal of Cardiology ◽

10.1016/j.ijcard.2021.10.157 ◽

2021 ◽

Author(s):

João Borges-Rosa ◽

Manuel Oliveira-Santos ◽

Rodolfo Silva ◽

Andreia Gomes ◽

José de Almeida ◽

...

Keyword(s):

Fdg Pet ◽

Cardiac Sarcoidosis ◽

European Population ◽

Single Centre ◽

Centre Study ◽

Single Centre Study ◽

18F Fdg

Download Full-text

The Role of 18F-FDG PET/CT in Follow up After Treatment in Childhood Langerhans Cell Histiocytosis.

The Egyptian Journal Nuclear Medicine ◽

10.21608/egyjnm.2019.106661 ◽

2019 ◽

Vol 19 (2) ◽

pp. 50-63

Author(s):

Ismail, A ◽

Abdel Gaid, S.

Keyword(s):

Fdg Pet ◽

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Pet Ct ◽

18F Fdg ◽

Fdg Pet Ct

Download Full-text

Incremental Role of 18F-FDG PET with contrast enhanced CT (PET-CECT) in detection of recurrence of carcinoma cervix

10.1055/s-0039-1685260 ◽

2016 ◽

Author(s):

S. Dash ◽

A. Goel ◽

S. Sogani

Keyword(s):

Fdg Pet ◽

Carcinoma Cervix ◽

Histopathological Correlation ◽

Contrast Enhanced Ct ◽

Contrast Enhanced ◽

Enhanced Ct ◽

Radiologic Imaging ◽

18F Fdg

Purpose: To evaluate the role of 18F-FDG PET with contrast enhanced CT (PET-CECT) in early detection of recurrence in follow up patients of carcinoma cervix. Methods: Patients with histopathologically proven carcinoma cervix who underwent chemotherapy, radiotherapy and/or surgery and on follow up were recruited in the study. Fifty-two patients underwent 18F-FDG PET-CECT for detection of recurrence. The median age was 51.5 (average = 53.4) years. PET-CECT studies were evaluated and analyzed separately by an experienced nuclear medicine physician and a radiologist independently. The physicians were blinded for the patient history. PET-CECT results were validated with histopathological correlation, conventional radiologic imaging/follow up PET-CECT study and clinical follow up. Results: Out of 52 patients, 34 patients were reported as positive for recurrence, 17 of these were having active local recurrence and 31 patients had regional lymph nodal metastases, 14 patients had distant metastases (out of them 6 patients had distant lymph node metastases, 6 had pulmonary metastases, 4 had skeletal metastases and two had liver metastases). Remaining 18 patients were reported as negative for recurrence. The lung was the most common site for distant metastasis. Patient were then further evaluated based on histopathological correlation, conventional radiologic imaging and follow up PET-CECT scan and five were found to be false positive and one patient was identified as false negative. The sensitivity, specificity, positive and negative predictive value were derived to be 96.7%, 77.3%, 85.3% and 94.4%, respectively. Accuracy was calculated to be 88.5%. Conclusions: 18F-FDG PET-CECT is a very useful non-invasive modality for the early detection of recurrence and metastatic workup in patients with carcinoma cervix with a very high sensitivity and negative predictive value. It is also useful in targeting biopsy sites in suspected cases of recurrence.

Download Full-text

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

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.345 ◽

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.

Download Full-text

Usefulness of 18f-FDG PET-CT in Staging, Restaging, and Response Assessment in Pediatric Rhabdomyosarcoma

Diagnostics ◽

10.3390/diagnostics10121112 ◽

2020 ◽

Vol 10 (12) ◽

pp. 1112

Author(s):

Davide Donner ◽

Paola Feraco ◽

Linda Meneghello ◽

Barbara Rombi ◽

Lorena Picori ◽

...

Keyword(s):

Fdg Pet ◽

Pediatric Patients ◽

Response Assessment ◽

Response To Therapy ◽

High Morbidity ◽

European Guidelines ◽

Pet Ct ◽

18F Fdg ◽

Fdg Pet Ct ◽

Clinical Advances

Rhabdomyosarcoma is the most common soft-tissue sarcoma of childhood. Despite clinical advances, subsets of these patients continue to suffer high morbidity and mortality rates associated with their disease. Following the European guidelines for 18F-FDG PET and PET-CT imaging in pediatric oncology, the routine use of 18F-FDG PET-CT may be useful for patients affected by rhabdomyosarcoma, in staging, in the evaluation of response to therapy, and for restaging/detection of relapse. The European Pediatric Protocols are very old, and for staging and restaging, they recommend only radionuclide bone scan. The 18F-FDG PET-CT exam is listed as an optional investigation prescribed according to local availability and local protocols in the investigations panel required at the end of the treatment. We present two cases highlighting the usefulness of 18F-FDG PET-CT in managing pediatric patients affected by rhabdomyosarcoma, providing some bibliographic references.

Download Full-text