Cardiac Sarcoidosis

Birnie, David H.; Nery, Pablo B.; Ha, Andrew C.T.; Beanlands, Rob

doi:10.1016/j.jacc.2016.03.605

Cited by 449 publications

(478 citation statements)

References 65 publications

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“…Cardiovascular manifestations were defined by the presence of the following disease classes in the primary or secondary diagnosis fields: conduction disorders, arrhythmias, heart failure, nonischemic cardiomyopathy, and pulmonary hypertension (Table S2). 18, 20, 21, 22 The NIS variables were used to identify demographic and baseline characteristics of sarcoidosis hospitalizations. Comorbidities used in our study were derived from Elixhauser method for quantification of comorbidities 14.…”

Section: Methodsmentioning

confidence: 99%

Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Patel

Kalra

Doshi

et al. 2018

JAHA

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BackgroundRecent trends of hospitalizations and in‐hospital mortality are not well defined in sarcoidosis. We examined aforementioned trends and prevalence of cardiovascular manifestations and explored rates of implantable cardioverter‐defibrillator implantation in hospitalizations with sarcoidosis.Methods and ResultsUsing data from the National Inpatient Sample, a retrospective population cohort from 2005 to 2014 was studied. To identify sarcoidosis, an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD‐9‐CM) diagnosis code was used. We excluded hospitalizations with myocardial infarction, coronary artery disease, and ischemic cardiomyopathy. Cardiovascular manifestations were defined by the presence of diagnosis codes for conduction disorders, arrhythmias, heart failure, nonischemic cardiomyopathy, and pulmonary hypertension. A total of 609 051 sarcoidosis hospitalizations were identified, with an age of 55±14 years, 67% women, and 50% black. The number of sarcoidosis hospitalizations increased from 2005 through 2014 (138 versus 175 per 100 000, P trend<0.001). We observed declining trends of unadjusted in‐hospital mortality (6.5 to 4.9 per 100 sarcoidosis hospitalizations, P trend<0.001). Overall ≈31% (n=188 438) of sarcoidosis hospitalizations had coexistent cardiovascular manifestations of one or more type. Heart failure (≈16%) and arrhythmias (≈15%) were the most prevalent cardiovascular manifestations. Rates of implantable cardioverter‐defibrillator placement were ≈7.5 per 1000 sarcoidosis hospitalizations (P trend=0.95) during the study period. Black race was associated with 21% increased risk of in‐hospital mortality (odds ratio, 1.21; 95% confidence interval, 1.16–1.27 [P<0.001]).ConclusionsSarcoidosis hospitalizations have increased over the past decade with a myriad of coexistent cardiovascular manifestations. Black race is a significant predictor of in‐hospital mortality, which is declining. Further efforts are needed to improve care in view of low implantable cardioverter‐defibrillator rates in sarcoidosis.

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Section: Methodsmentioning

confidence: 99%

Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Patel

Kalra

Doshi

et al. 2018

JAHA

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confidence: 99%

“…Cardiac involvement is clinically manifest in just 5% to 10% of sarcoidosis patients, but can be identified at autopsy in approximately 25% and is the most frequent cause of death (1,2). The accurate diagnosis of subclinical but active cardiac sarcoidosis (aCS) is, therefore, important (1); however, a gold standard assessment remains lacking. The yield of endomyocardial biopsy is low due to the patchy distribution of myocardial disease (3), the Japanese Ministry of Health and Welfare criteria (4) lack sensitivity, and echocardiographic findings of regional wall thickening/thinning or motion abnormalities are neither specific nor sensitive (1).…”

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confidence: 99%

Hybrid Magnetic Resonance Imaging and Positron Emission Tomography With Fluorodeoxyglucose to Diagnose Active Cardiac Sarcoidosis

Dweck

Abgral

Trivieri

et al. 2018

JACC: Cardiovascular Imaging

165

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OBJECTIVES The purpose of this study was to explore the diagnostic usefulness of hybrid cardiac magnetic resonance (CMR) and positron emission tomography (PET) using 18F-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 (Ki) 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 Ki 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 < 0.001), demonstrating an area under the curve of 0.98 on receiver-operating characteristic analysis for the detection of aCS, with an optimal maximum target-to-normal myocardium ratio threshold of 1.2 (Youden index: 0.94). 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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“…40% already had an implantable cardiac defibrillator prior to PET/CT, mean ejection fraction was 47 ± 16%, and JMHWG criteria turned positive in 34% of study population. In this line of thought, a recent review that included eight studies on prevalence and prognosis of clinically silent CS reported a more favourable course in clinical silent CS in five out of these eight studies: within a mean follow‐up of 23 months, no adverse cardiac event like ventricular arrhythmia or death was reported 24. It illustrates that differences in cardiac symptomatology of study population may influence adverse events' occurrence.…”

Section: Discussionmentioning

confidence: 98%

Cardiovascular magnetic resonance imaging and clinical performance of somatostatin receptor positron emission tomography in cardiac sarcoidosis

et al. 2017

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AimsCardiac affection constitutes a major limiting condition in systemic sarcoidosis. The primary objective of this study was to investigate the persistence rate of cardiac sarcoid involvement by cardiovascular magnetic resonance (CMR) imaging in patients diagnosed with cardiac sarcoidosis (CS). Moreover, we examined the additional insights into myocardial damage's characteristics gained by somatostatin receptor scintigraphy.Methods and resultsIn a pilot study, we had previously identified cardiac involvement—diagnosed by CMR imaging—to be present in 29 of 188 patients (15.4%) with histologically proven, extra‐CS. Out of these initial 29 CS‐positive patients, 27 patients (49.9 ± 11.8 years, 59.3% male) were presently re‐examined and underwent a second CMR study and complementary standard clinical testing. Somatostatin receptor scintigraphy using the ligand 68Ga‐DOTATOC was additionally performed when clinically indicated (17 patients). Within a median follow‐up period of 2.6 years, none of the initial 29 patients deceased or experienced aborted sudden cardiac death. However, two patients developed third‐degree atrioventricular block that required device therapy. Among the 27 re‐examined CS patients, pathological CMR findings persisted in 14 of 27 patients (51.9%). CS remission was primarily due to a resolution of acute inflammatory processes. 68Ga‐DOTATOC positron emission tomography/computed tomography (PET/CT) identified one patient with regions of raised tracer uptake that concorded with acute inflammatory changes, as assessed by CMR; this patient received no immunosuppressive medication at the time of PET/CT execution.ConclusionsWithin follow‐up, CS persisted in barely half the patients, and the patients were not afflicted with cardiac death. Additional 68Ga‐DOTATOC PET/CT allowed for visualization of acute myocardial inflammation.

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Cardiac Sarcoidosis

Cited by 449 publications

References 65 publications

Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Hybrid Magnetic Resonance Imaging and Positron Emission Tomography With Fluorodeoxyglucose to Diagnose Active Cardiac Sarcoidosis

Cardiovascular magnetic resonance imaging and clinical performance of somatostatin receptor positron emission tomography in cardiac sarcoidosis

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