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David H. Birnie - One of the best experts on this subject based on the ideXlab platform.
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Cardiac Sarcoidosis multi center randomized controlled trial chasm cs rct
American Heart Journal, 2020Co-Authors: David H. Birnie, Pablo B. Nery, Rob S. Beanlands, Shawn D Aaron, Daniel A Culver, Robert A Dekemp, Lorne J Gula, Jeffery S Healey, Yuko Inoue, Mark A JudsonAbstract:Background Approximately 5% of patients with Sarcoidosis have clinically manifest Cardiac involvement. Clinical features of Cardiac Sarcoidosis are dependent on the location, extent, and activity of the disease. First line therapy is usually with prednisone and this is recommended based on clinician experience, expert opinion and small observational cohorts. There are no published clinical trials in Cardiac Sarcoidosis and multiple experts in the field have called for randomized clinical trials to answer important patient care questions. Corticosteroid are associated with multiple adverse effects including hypertension, diabetes, weight gain, osteoporosis, and increased risk of infections. In contrast Methotrexate is generally well tolerated and is increasingly used in other forms of Sarcoidosis. Objectives The Cardiac Sarcoidosis Multi-Center Randomized Controlled Trial (CHASM CS-RCT; NCT03593759 ) is a multicenter randomized controlled trial designed to evaluate the optimal initial treatment strategy for patients with active Cardiac Sarcoidosis. We hypothesize that (1) a low dose prednisone/methotrexate combination will have non-inferior efficacy to standard dose prednisone and that (2) the low dose prednisone/ methotrexate combination will result in significantly better quality of life than standard dose prednisone, as a result of reduced burden of side effects. Methods/design Eligible study subjects will have active clinically manifest Cardiac Sarcoidosis presenting with one or more of the following clinical findings: advanced conduction system disease, significant sinus node dysfunction, non-sustained or sustained ventricular arrhythmia, left ventricular dysfunction or right ventricular dysfunction. Subjects will be randomized in a 1:1 ratio to prednisone 0.5 mg/kg/day for 6 months (maximum dose 30 mg daily) OR to prednisone 20 mg daily for 1 month, then 10 mg daily for 1 month, then 5 mg daily for one month then stop AND methotrexate 15–20 mg once weekly for 6 months. The primary endpoint is summed perfusion rest score on 6-month PET (blinded core-lab review). The summed perfusion rest score is measure of myocardial fibrosis/scar. The design is non-inferiority with a sample size of 97 per group. Discussion Given the multiorgan system potential adverse side effects of prednisone, proving noninferiority of an alternate regimen would be sufficient to make the alternative compare favorably to standard dose steroids. This is the first ever clinical trial in Cardiac Sarcoidosis and thus in addition to the listed goals of the trial, we will also establish a multi-center, multinational Cardiac Sarcoidosis clinical trials network. Such a collaborative infrastructure will enable a new era of high quality data to guide physicians when treating Cardiac Sarcoidosis patients.
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molecular imaging of Cardiac Sarcoidosis
Current Cardiovascular Imaging Reports, 2018Co-Authors: Patrick Martineau, David H. Birnie, Matthieu Pelletiergalarneau, Daniel Juneau, Eugene Leung, Rob S. BeanlandsAbstract:Cardiac involvement is increasingly being recognized as a cause of significant morbidity and mortality in patients with Sarcoidosis. Unfortunately, imaging Cardiac Sarcoidosis (CS) remains a challenge and often relies on advanced imaging modalities. The aim of this review is to provide an up-to-date overview of the role of molecular imaging for the diagnosis of Cardiac Sarcoidosis, with particular emphasis on the use of FDG and non-FDG PET. In the last few years, FDG-PET has established itself as an accurate imaging study for the assessment of CS. New diagnostic criteria have recently appeared which incorporate FDG-PET findings in the diagnostic workup for CS. In addition, a joint SNMMI-ASNC Expert Consensus document has appeared on the role of FDG-PET in CS. Recent advances in the field have focused on the role of PET for diagnosis, assessment of therapy, and use of non-FDG tracers to image myocardial inflammation. The role of PET is rapidly evolving but is now firmly established as an important component in the imaging assessment of CS.
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corticosteroid therapy for Cardiac Sarcoidosis a systematic review
Canadian Journal of Cardiology, 2013Co-Authors: Mouhannad M Sadek, David H. Birnie, Rob S. Beanlands, Derek Yung, Pablo B. NeryAbstract:Abstract Background There are no published clinical consensus guidelines or systematic evaluation supporting the use of corticosteroids for the treatment of Cardiac Sarcoidosis. The purpose of this study was to systematically review the published data on corticosteroid treatment of Cardiac Sarcoidosis. Methods Studies were identified from MEDLINE, EMBASE, Cochrane Controlled Trials Register, Cochrane Database of Systematic Reviews, and National Institutes of Health Clinical Trials.gov database. The quality of included articles was rated using Scottish Intercollegiate Guidelines Network 50. Outcomes examined were atrioventricular (AV) conduction, left ventricular function, ventricular arrhythmias, and mortality. Results A total of 1491 references were retrieved and 10 publications met the inclusion criteria. There were no randomized trials and all publications were of poor to fair quality. In the 10 reports, 257 patients received corticosteroids and 42 patients did not. There were 57 patients with AV conduction disease treated with corticosteroids, with 27/57 (47.4%) improving. In contrast, 16 patients were not treated with corticosteroids and 0/16 improved. Four publications reported on left ventricular function recovery, 2 reported on ventricular arrhythmia burden, and 9 reported on mortality. However, the data quality were too limited to draw conclusions for any of these outcomes. Conclusions Our systematic review identified 10 publications reporting outcomes after corticosteroid therapy. The best data relates to AV conduction recovery and corticosteroids appeared to be beneficial. It is not possible to draw clear conclusions about the utility of corticosteroids for the other outcomes. There is a clear need for large multicentre prospective registries and trials in this patient population.
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Isolated Cardiac Sarcoidosis: Establishing the Diagnosis With Electroanatomic Mapping-Guided Endomyocardial Biopsy
The Canadian journal of cardiology, 2013Co-Authors: Pablo B. Nery, Arieh Keren, Jeff S. Healey, Eugene Leug, Rob S. Beanlands, David H. BirnieAbstract:Diagnosing isolated Cardiac Sarcoidosis can be challenging, and requires a high index of suspicion. We report a case of a young woman who presented with sustained ventricular tachycardia, intermittent atrioventricular block and epsilon wave on electrocardiogram. Although the patient fulfilled Task Force criteria for arrhythmogenic right ventricular cardiomyopathy, Sarcoidosis was suspected because of the presence of intermittent atrioventricular block. As illustrated in this report, the use of electroanatomic mapping-guided endomyocardial biopsy can be decisive in achieving the diagnosis and is a valuable approach in cases of suspected isolated Cardiac Sarcoidosis.
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the use of 18f fdg pet in the diagnosis of Cardiac Sarcoidosis a systematic review and metaanalysis including the ontario experience
The Journal of Nuclear Medicine, 2012Co-Authors: George Youssef, Robert A Dekemp, Eugene Leung, Ilias Mylonas, Pablo Nery, Kathryn Williams, Gerald Wisenberg, Karen Y Gulenchyn, Jean N Dasilva, David H. BirnieAbstract:UNLABELLED: Cardiac Sarcoidosis is a potentially fatal complication of Sarcoidosis. The 1993 guidelines of the Ministry of Health, Labour, and Welfare (MHLW) of Japan have been used as the diagnostic gold standard and for comparison with imaging modalities. (18)F-FDG PET is not currently included in the guidelines. However, studies have shown promising data using (18)F-FDG PET. We conducted a systematic review of studies that evaluated the accuracy of (18)F-FDG PET for the diagnosis of Cardiac Sarcoidosis compared with MHLW guidelines. Data from a prospective Ontario provincial registry are also reported and included in the metaanalysis. METHODS: PubMed, Embase, and the Cochrane Central Register of Controlled Trials were searched for studies that satisfied predetermined criteria. Quality evaluation using the Quality Assessment for Diagnostic Accuracy Studies was performed by 2 independent masked observers. Data were extracted and analyzed to measure study-specific and pooled accuracy for (18)F-FDG PET compared with the MHLW as the reference. RESULTS: A total of 519 titles was identified; 7 studies, including the Ontario registry, were selected for inclusion. Metaanalysis of these 7 studies was conducted, with a total of 164 patients, most of whom had been diagnosed with systemic Sarcoidosis. The prevalence of Cardiac Sarcoidosis was 50% in the whole population. Pooled estimates for (18)F-FDG PET yielded 89% sensitivity (95% confidence interval [CI], 79%-96%), 78% specificity (95% CI, 68%-86%), a 4.1 positive likelihood ratio (95% CI, 1.7-10), and a 0.19 negative likelihood ratio (95% CI, 0.1-0.4). The overall diagnostic odds ratio was 25.6 (95% CI, 7.3-89.5), and the area under the summary receiver operator characteristic curve was 93% ± 3.5. The Ontario study yielded sensitivity and specificity of 79% and 70%, respectively. CONCLUSION: The high diagnostic accuracy determined for (18)F-FDG PET in this metaanalysis suggests potential value for diagnosis of Cardiac Sarcoidosis compared with the MHLW guidelines. These results may affect patient care by providing supportive evidence for more effective use of (18)F-FDG PET in the diagnosis of Cardiac Sarcoidosis. Large-scale multicenter studies are required to further evaluate this role.
Michael T Osborne - One of the best experts on this subject based on the ideXlab platform.
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Cardiac Sarcoidosis initially diagnosed as spontaneous coronary artery dissection
Case Reports, 2021Co-Authors: Hadil Zureigat, Rachel C Frank, Viral Shah, Vladislav V Makarenko, William J Hucker, Malissa J Wood, Michael T OsborneAbstract:We present the case of a woman who developed presumed spontaneous coronary artery dissection of a septal branch. She later developed high-grade atrioventricular block that led to a diagnosis of Cardiac Sarcoidosis involving the interventricular septum. This case illustrates a rare and challenging presentation of Cardiac Sarcoidosis. (Level of Difficulty: Beginner.).
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isolated Cardiac Sarcoidosis a focused review of an under recognized entity
Journal of Nuclear Cardiology, 2018Co-Authors: David R Okada, Paco E Bravo, Michael T Osborne, Sharmila Dorbala, Hicham Skali, Tomas Vita, Vikram Agarwal, Viviany R Taqueti, Panithaya Chareonthaitawee, Garrick C StewartAbstract:There is accumulating evidence for the existence of a phenotype of isolated Cardiac Sarcoidosis (ICS), or Sarcoidosis that only involves the heart. In the absence of biopsy-confirmed Cardiac Sarcoidosis (CS), existing diagnostic criteria require the presence of extra-Cardiac Sarcoidosis as an inclusion criterion for the diagnosis of CS. Consequently, in the absence of a positive endomyocardial biopsy, ICS is not diagnosable by current guidelines. Therefore, there is uncertainty regarding the epidemiology, pathobiology, clinical characteristics, prognosis, and optimal treatment of ICS. This review will summarize the available data related to the prevalence and prognosis of ICS and will discuss challenges surrounding the diagnosis and management of this under-recognized entity.
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Cardiac Sarcoidosis state of the art review
Cardiovascular diagnosis and therapy, 2016Co-Authors: Edward Hulten, Michael T Osborne, Marcio Sommer Bittencourt, Saira Aslam, Siddique Abbasi, Ron BlanksteinAbstract:Sarcoidosis is a multi-system inflammatory disorder of unknown etiology resulting in formation of non-caseating granulomas. Cardiac involvement—which is associated with worse prognosis—has been detected in approximately 25% of individuals based on autopsy or Cardiac imaging studies. Nevertheless, the diagnosis of Cardiac Sarcoidosis is challenging due to the low yield of endomyocardial biopsy, and the limited accuracy of various clinical criteria. Thus, no gold standard diagnostic criterion exists. This review will summarize the pathophysiology, diagnosis, and treatment of Cardiac Sarcoidosis with a focus on advanced cardiovascular imaging, We review the evidence to support a role for Cardiac magnetic resonance (CMR) imaging in the initial evaluation of selected patients with suspected Cardiac Sarcoidosis, with Cardiac positron emission tomography (PET) as an alternative or complementary initial diagnostic test in a subgroup of patients in whom CMR may be contra-indicated or when CMR is negative with continued clinical concern for myocardial inflammation. In addition to the diagnostic value of these tests, CMR and PET are also useful in identifying patients who have higher risk of adverse events such as ventricular tachycardia or death, in whom preventive therapies such as defibrillators should be more strongly considered. Although no randomized controlled trials for treatment of Cardiac Sarcoidosis exist, immunosuppressive therapy is often used. We review emerging evidence regarding the use of Cardiac PET to identify and quantity the amount of myocardial inflammation as well as to guide the use of immunotherapy. Future studies are needed to determine the benefit of imaging guided therapies aimed at improving patient outcomes.
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reduction in 18f fluorodeoxyglucose uptake on serial Cardiac positron emission tomography is associated with improved left ventricular ejection fraction in patients with Cardiac Sarcoidosis
Journal of Nuclear Cardiology, 2014Co-Authors: Michael T Osborne, Edward Hulten, Avinainder Singh, Marcio Sommer Bittencourt, Garrick C Stewart, Jon Hainer, Venkatesh L Murthy, Hicham Skali, Alfonso H Waller, Sharmila DorbalaAbstract:Background Cardiac positron emission tomography (PET) using 18F-fluorodeoxyglucose (FDG) has been used to diagnose and monitor Cardiac Sarcoidosis (CS). It is not known whether a reduction in myocardial inflammation, as measured by FDG uptake, is associated with improvement in LV ejection fraction (EF).
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Cardiac positron emission tomography enhances prognostic assessments of patients with suspected Cardiac Sarcoidosis
Journal of the American College of Cardiology, 2012Co-Authors: Ron Blankstein, Michael T Osborne, Venkatesh L Murthy, Alfonso H Waller, Masanao Naya, Chun K Kim, Pedram Kazemian, Michifumi Tokuda, Hicham SkaliAbstract:Objectives This study sought to relate imaging findings on positron emission tomography (PET) to adverse Cardiac events in patients referred for evaluation of known or suspected Cardiac Sarcoidosis. Background Although Cardiac PET is commonly used to evaluate patients with suspected Cardiac Sarcoidosis, the relationship between PET findings and clinical outcomes has not been reported. Methods We studied 118 consecutive patients with no history of coronary artery disease, who were referred for PET, using [18F]fluorodeoxyglucose (FDG) to assess for inflammation and rubidium-82 to evaluate for perfusion defects (PD), following a high-fat/low-carbohydrate diet to suppress normal myocardial glucose uptake. Blind readings of PET data categorized Cardiac findings as normal, positive PD or FDG, positive PD and FDG. Images were also used to identify whether findings of extra-Cardiac Sarcoidosis were present. Adverse events (AE)—death or sustained ventricular tachycardia (VT)—were ascertained by electronic medical records, defibrillator interrogation, patient questionnaires, and telephone interviews. Results Among the 118 patients (age 52 ± 11 years; 57% males; mean ejection fraction: 47 ± 16%), 47 (40%) had normal and 71 (60%) had abnormal Cardiac PET findings. Over a median follow-up of 1.5 years, there were 31 (26%) adverse events (27 VT and 8 deaths). Cardiac PET findings were predictive of AE, and the presence of both a PD and abnormal FDG (29% of patients) was associated with hazard ratio of 3.9 (p Conclusions The presence of focal PD and FDG uptake on Cardiac PET identifies patients at higher risk of death or VT. These findings offer prognostic value beyond Japanese Ministry of Health and Welfare clinical criteria, the presence of extra-Cardiac Sarcoidosis and LVEF.
Leslie T Cooper - One of the best experts on this subject based on the ideXlab platform.
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advances in imaging for diagnosis and management of Cardiac Sarcoidosis
European Journal of Echocardiography, 2015Co-Authors: Niti R Aggarwal, David Snipelisky, Phillip M Young, Bernard J Gersh, Leslie T Cooper, Panithaya ChareonthaitaweeAbstract:Sarcoidosis is a multisystem granulomatous disorder with a high prevalence of Cardiac involvement. Cardiac Sarcoidosis (CS) may be life threatening due to end-stage cardiomyopathy and sudden Cardiac death. The frequent absence of specific symptoms and lack of a diagnostic 'gold standard' pose challenges in the diagnosis of CS. Endomyocardial biopsy, although specific, has an unacceptably low sensitivity. Non-invasive Cardiac imaging has a huge role in the assessment of patients with known or suspected CS. This comprehensive review compares the diagnostic accuracy, along with advantages and disadvantages, of established and emerging imaging modalities for CS.
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idiopathic giant cell myocarditis and Cardiac Sarcoidosis
Heart Failure Reviews, 2013Co-Authors: Lori A Blauwet, Leslie T CooperAbstract:Idiopathic giant cell myocarditis (GCM) and Cardiac Sarcoidosis (CS) are rare disorders that cause cardiomyopathy, often with ventricular arrhythmias or heart block. Infection, autoimmune processes, and genetics have all been implicated in the pathogenesis of these diseases, but the etiology for both diseases is likely a complex multifactorial process. Both GCM and CS are generally progressive despite treatment with standard heart failure and arrhythmia therapies. Making the diagnosis of GCM or CS on initial clinical presentation is possible in only a small percentage of patients, so myocardial tissue diagnosis is required. The use of multiple noninvasive imaging modalities may aid in diagnosis and assessment of response to treatment. Establishing the diagnosis of GCM or CS early is crucial, as tailored immunosuppressive treatment may significantly alter the clinical course of these patients. The prognosis of patients with GCM is poor, while the prognosis for patients with CS varies according to degree of left ventricular dysfunction.
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a clinical and histopathologic comparison of Cardiac Sarcoidosis and idiopathic giant cell myocarditis
Journal of the American College of Cardiology, 2003Co-Authors: Yuji Okura, Joshua M Hare, Makoto Kodama, Gerald J Berry, Henry D Tazelaar, Kent R Bailey, Leslie T CooperAbstract:Abstract Objectives The goal of this study was to determine the prognostic value of clinical data available at presentation and histology in Cardiac Sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM). Background The prognosis of patients with nonischemic cardiomyopathy is partly dependent on the histologic diagnosis. Survival in IGCM is poor. The prognosis of a histologically related entity, Cardiac Sarcoidosis (CS), is less well established, and the prognostic value of the distinction between CS and IGCM on endomyocardial biopsy (EMB) is unknown. Methods We identified 115 patients from the Multicenter IGCM Registry with CS (n = 42) and IGCM (n = 73). We compared the clinical data for these two groups using Cox proportional-hazards models to assess the association between histologic diagnosis and survival. In order to determine whether histologic features could reliably differentiate these two entities, two Cardiac pathologists semiquantitatively graded the inflammatory infiltrate components and compared the results between groups. Results Black race was more frequent in the CS group (31% vs. 4%, p Conclusions Transplant-free survival is better for patients with CS than for IGCM diagnosed by EMB. Presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS.
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a clinical and histopathologic comparison of Cardiac Sarcoidosis and idiopathic giant cell myocarditis
Journal of the American College of Cardiology, 2003Co-Authors: Yuji Okura, Joshua M Hare, Makoto Kodama, Gerald J Berry, Henry D Tazelaar, Kent R Bailey, William G Dec, Leslie T CooperAbstract:OBJECTIVES The goal of this study was to determine the prognostic value of clinical data available at presentation and histology in Cardiac Sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM). BACKGROUND The prognosis of patients with nonischemic cardiomyopathy is partly dependent on the histologic diagnosis. Survival in IGCM is poor. The prognosis of a histologically related entity, Cardiac Sarcoidosis (CS), is less well established, and the prognostic value of the distinction between CS and IGCM on endomyocardial biopsy (EMB) is unknown. METHODS We identified 115 patients from the Multicenter IGCM Registry with CS (n = 42) and IGCM (n = 73). We compared the clinical data for these two groups using Cox proportional-hazards models to assess the association between histologic diagnosis and survival. In order to determine whether histologic features could reliably differentiate these two entities, two Cardiac pathologists semiquantitatively graded the inflammatory infiltrate components and compared the results between groups. RESULTS Black race was more frequent in the CS group (31% vs. 4%, p < 0.0001). Syncope and atrioventricular block were also more frequently observed in CS than IGCM (31% vs. 5%, p = 0.0002 and 50% vs. 15%, p < 0.0001, respectively). Left-sided heart failure was more common in IGCM (40% vs. 64%, p = 0.013). In CS patients diagnosed by EMB, the five-year transplant-free survival after diagnosis was 69.8% versus 21.9% for IGCM (p < 0.0001, log-rank test). In multivariate models, presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS. CONCLUSIONS Transplant-free survival is better for patients with CS than for IGCM diagnosed by EMB. Presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS.
Helena L Chang - One of the best experts on this subject based on the ideXlab platform.
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hybrid magnetic resonance imaging and positron emission tomography with fluorodeoxyglucose to diagnose active Cardiac Sarcoidosis
Jacc-cardiovascular Imaging, 2018Co-Authors: Marc R Dweck, Ronan Abgral, Maria Giovanna Trivieri, Philip M Robson, Nicolas A Karakatsanis, Venkatesh Mani, Anna Palmisano, Marc A Miller, Anuradha Lala, Helena L ChangAbstract:Abstract Objectives The purpose of this study was to explore the diagnostic usefulness of hybrid Cardiac magnetic resonance (CMR) and positron emission tomography (PET) using 18 F-fluorodeoxyglucose (FDG) for active Cardiac Sarcoidosis. Background Active Cardiac Sarcoidosis (aCS) is underdiagnosed and has a high mortality. Methods Patients with clinical suspicion of aCS underwent hybrid CMR/PET with late gadolinium enhancement (LGE) and FDG to assess the pattern of injury and disease activity, respectively. Patients were categorized visually as magnetic resonance (MR)+PET+ (characteristic LGE aligning exactly with increased FDG uptake), MR+PET− (characteristic LGE but no increased FDG), MR−PET− (neither characteristic LGE nor increased FDG), and MR−PET+ (increased FDG uptake in absence of characteristic LGE) and further characterized as aCS+ (MR+PET+) or aCS− (MR+PET−, MR−PET−, MR−PET+). FDG uptake was quantified using maximum target-to-normal-myocardium ratio and the net uptake rate ( K i ) from dynamic Patlak analysis. Receiver-operating characteristic methods were used to identify imaging biomarkers for aCS. FDG PET was assessed using computed tomography/PET in 19 control subjects with healthy myocardium. Results A total of 25 patients (12 males; 54.9 ± 9.8 years of age) were recruited prospectively; 8 were MR+PET+, suggestive of aCS; 1 was MR+PET−, consistent with inactive Cardiac Sarcoidosis; and 8 were MR−PET−, with no imaging evidence of Cardiac Sarcoidosis. Eight patients were MR−PET+ (6 with global myocardial FDG uptake, 2 with focal-on-diffuse uptake); they demonstrated distinct K i values and hyperintense maximum standardized uptake value compared with MR+PET+ patients. Similar hyperintense patterns of global (n = 9) and focal-on-diffuse (n = 2) FDG uptake were also observed in control patients, suggesting physiological myocardial uptake. Maximum target-to-normal-myocardium ratio values were higher in the aCS+ group (p Conclusions CMR/PET imaging holds major promise for the diagnosis of aCS, providing incremental information about both the pattern of injury and disease activity in a single scan. (In Vivo Molecular Imaging [MRI] of Atherothrombotic Lesions; NCT01418313)
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hybrid magnetic resonance imaging and positron emission tomography with fluorodeoxyglucose to diagnose active Cardiac Sarcoidosis
Jacc-cardiovascular Imaging, 2018Co-Authors: Marc R Dweck, Ronan Abgral, Maria Giovanna Trivieri, Philip M Robson, Nicolas A Karakatsanis, Venkatesh Mani, Anna Palmisano, Marc A Miller, Anuradha Lala, Helena L ChangAbstract:Abstract Objectives The purpose of this study was to explore the diagnostic usefulness of hybrid Cardiac magnetic resonance (CMR) and positron emission tomography (PET) using 18 F-fluorodeoxyglucose (FDG) for active Cardiac Sarcoidosis. Background Active Cardiac Sarcoidosis (aCS) is underdiagnosed and has a high mortality. Methods Patients with clinical suspicion of aCS underwent hybrid CMR/PET with late gadolinium enhancement (LGE) and FDG to assess the pattern of injury and disease activity, respectively. Patients were categorized visually as magnetic resonance (MR)+PET+ (characteristic LGE aligning exactly with increased FDG uptake), MR+PET− (characteristic LGE but no increased FDG), MR−PET− (neither characteristic LGE nor increased FDG), and MR−PET+ (increased FDG uptake in absence of characteristic LGE) and further characterized as aCS+ (MR+PET+) or aCS− (MR+PET−, MR−PET−, MR−PET+). FDG uptake was quantified using maximum target-to-normal-myocardium ratio and the net uptake rate ( K i ) from dynamic Patlak analysis. Receiver-operating characteristic methods were used to identify imaging biomarkers for aCS. FDG PET was assessed using computed tomography/PET in 19 control subjects with healthy myocardium. Results A total of 25 patients (12 males; 54.9 ± 9.8 years of age) were recruited prospectively; 8 were MR+PET+, suggestive of aCS; 1 was MR+PET−, consistent with inactive Cardiac Sarcoidosis; and 8 were MR−PET−, with no imaging evidence of Cardiac Sarcoidosis. Eight patients were MR−PET+ (6 with global myocardial FDG uptake, 2 with focal-on-diffuse uptake); they demonstrated distinct K i values and hyperintense maximum standardized uptake value compared with MR+PET+ patients. Similar hyperintense patterns of global (n = 9) and focal-on-diffuse (n = 2) FDG uptake were also observed in control patients, suggesting physiological myocardial uptake. Maximum target-to-normal-myocardium ratio values were higher in the aCS+ group (p Conclusions CMR/PET imaging holds major promise for the diagnosis of aCS, providing incremental information about both the pattern of injury and disease activity in a single scan. (In Vivo Molecular Imaging [MRI] of Atherothrombotic Lesions; NCT01418313)
Ron Blankstein - One of the best experts on this subject based on the ideXlab platform.
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Advanced cardiovascular imaging for the evaluation of Cardiac Sarcoidosis
Journal of Nuclear Cardiology, 2018Co-Authors: Paco E Bravo, Amitoj Singh, Marcelo F Di Carli, Ron BlanksteinAbstract:Cardiac Sarcoidosis (CS) remains an intriguing infiltrating disorder and one of the most important forms of inflammatory cardiomyopathy. Identification of patients with CS is of extreme importance because they are at higher risk of sudden death, and heart-failure progression. And while it remains a diagnostic conundrum, a great amount of experience has been accumulated over the last decade with the advent of fluorine-18 fluorodeoxyglucose positron emission tomography and Cardiac magnetic resonance with late gadolinium enhancement imaging. They have both proven to be advanced imaging techniques that provide important, and often complementary, diagnostic and prognostic information for the management of CS. However, they have also shown to have limitations, and, thus, there is a continued need for developing more specific imaging probes for identifying Cardiac inflammation. The aim of the present manuscript is to provide the reader with a better understanding of the histopathology of the disease, how this potentially relates to noninvasive imaging detection, and the best strategies available for the diagnosis and management of patients with CS.
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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
European Journal of Echocardiography, 2017Co-Authors: Riemer H J A Slart, Ron Blankstein, Andor W J M Glaudemans, Patrizio Lancellotti, Fabien Hyafil, Ronald G Schwartz, Wael A Jaber, Raymond R Russell, Alessia Gimelli, Francois RouzetAbstract:This joint position paper illustrates the role and the correct use of echocardiography, radionuclide imaging with F-18-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.
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evaluation of known or suspected Cardiac Sarcoidosis
Circulation-cardiovascular Imaging, 2016Co-Authors: Ron Blankstein, Alfonso H WallerAbstract:Sarcoidosis is a multisystem disorder of unknown cause, and Cardiac Sarcoidosis affects at least 25% of patients and accounts for substantial mortality and morbidity from this disease. Cardiac Sarcoidosis may present with heart failure, left ventricular systolic dysfunction, AV block, atrial or ventricular arrhythmias, and sudden Cardiac death. Cardiac involvement can be challenging to detect and diagnose because of the focal nature of the disease, as well as the fact that clinical criteria have limited diagnostic accuracy. Nevertheless, the diagnosis of Cardiac Sarcoidosis can be enhanced by integrating both clinical and imaging findings. This article reviews the various roles that different imaging modalities provide in the evaluation and management of patients with known or suspected Cardiac Sarcoidosis.
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Cardiac Sarcoidosis state of the art review
Cardiovascular diagnosis and therapy, 2016Co-Authors: Edward Hulten, Michael T Osborne, Marcio Sommer Bittencourt, Saira Aslam, Siddique Abbasi, Ron BlanksteinAbstract:Sarcoidosis is a multi-system inflammatory disorder of unknown etiology resulting in formation of non-caseating granulomas. Cardiac involvement—which is associated with worse prognosis—has been detected in approximately 25% of individuals based on autopsy or Cardiac imaging studies. Nevertheless, the diagnosis of Cardiac Sarcoidosis is challenging due to the low yield of endomyocardial biopsy, and the limited accuracy of various clinical criteria. Thus, no gold standard diagnostic criterion exists. This review will summarize the pathophysiology, diagnosis, and treatment of Cardiac Sarcoidosis with a focus on advanced cardiovascular imaging, We review the evidence to support a role for Cardiac magnetic resonance (CMR) imaging in the initial evaluation of selected patients with suspected Cardiac Sarcoidosis, with Cardiac positron emission tomography (PET) as an alternative or complementary initial diagnostic test in a subgroup of patients in whom CMR may be contra-indicated or when CMR is negative with continued clinical concern for myocardial inflammation. In addition to the diagnostic value of these tests, CMR and PET are also useful in identifying patients who have higher risk of adverse events such as ventricular tachycardia or death, in whom preventive therapies such as defibrillators should be more strongly considered. Although no randomized controlled trials for treatment of Cardiac Sarcoidosis exist, immunosuppressive therapy is often used. We review emerging evidence regarding the use of Cardiac PET to identify and quantity the amount of myocardial inflammation as well as to guide the use of immunotherapy. Future studies are needed to determine the benefit of imaging guided therapies aimed at improving patient outcomes.
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Cardiac positron emission tomography enhances prognostic assessments of patients with suspected Cardiac Sarcoidosis
Journal of the American College of Cardiology, 2012Co-Authors: Ron Blankstein, Michael T Osborne, Venkatesh L Murthy, Alfonso H Waller, Masanao Naya, Chun K Kim, Pedram Kazemian, Michifumi Tokuda, Hicham SkaliAbstract:Objectives This study sought to relate imaging findings on positron emission tomography (PET) to adverse Cardiac events in patients referred for evaluation of known or suspected Cardiac Sarcoidosis. Background Although Cardiac PET is commonly used to evaluate patients with suspected Cardiac Sarcoidosis, the relationship between PET findings and clinical outcomes has not been reported. Methods We studied 118 consecutive patients with no history of coronary artery disease, who were referred for PET, using [18F]fluorodeoxyglucose (FDG) to assess for inflammation and rubidium-82 to evaluate for perfusion defects (PD), following a high-fat/low-carbohydrate diet to suppress normal myocardial glucose uptake. Blind readings of PET data categorized Cardiac findings as normal, positive PD or FDG, positive PD and FDG. Images were also used to identify whether findings of extra-Cardiac Sarcoidosis were present. Adverse events (AE)—death or sustained ventricular tachycardia (VT)—were ascertained by electronic medical records, defibrillator interrogation, patient questionnaires, and telephone interviews. Results Among the 118 patients (age 52 ± 11 years; 57% males; mean ejection fraction: 47 ± 16%), 47 (40%) had normal and 71 (60%) had abnormal Cardiac PET findings. Over a median follow-up of 1.5 years, there were 31 (26%) adverse events (27 VT and 8 deaths). Cardiac PET findings were predictive of AE, and the presence of both a PD and abnormal FDG (29% of patients) was associated with hazard ratio of 3.9 (p Conclusions The presence of focal PD and FDG uptake on Cardiac PET identifies patients at higher risk of death or VT. These findings offer prognostic value beyond Japanese Ministry of Health and Welfare clinical criteria, the presence of extra-Cardiac Sarcoidosis and LVEF.