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Pubblicazioni recenti - cardiac sarcoidosis
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Patient-reported outcome measures in systemic sclerosis-related interstitial lung disease for clinical practice and clinical trials.
J Scleroderma Relat Disord2020 Mar;5(2 Suppl):48-60. doi: 10.1177/2397198320904178.
Saketkoo Lesley Ann, Scholand Mary Beth, Lammi Matthew R, Russell Anne-Marie,
Abstract
Systemic sclerosis (SSc) is a progressive vasculopathic, fibrosing autoimmune condition, portending significant mortality; wherein interstitial lung disease (ILD) is the leading cause of death. Although lacking a definitive cure, therapeutics for (SSc-ILD) that stave progression exist with further promising primary and adjuvant compounds in development, as well as interventions to reduce symptom burden and increase quality of life. To date, there has been a significant but varied history related to systemic sclerosis-related interstitial lung disease trial design and endpoint designation. This is especially true of endpoints measuring patient-reported perceptions of efficacy and tolerability. This article describes the underpinnings and complexity of the science, methodology, and current state of patient-reported outcome measures used in (SSc-ILD) systemic sclerosis-related interstitial lung disease in clinical practice and trials.
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A positive Tc-99m PYP scan in a patient with cardiac sarcoidosis.
J Nucl Cardiol2020 May;():. doi: 10.1007/s12350-020-02158-5.
El-Tallawi K Carlos, Parikh Roosha, Nabi Faisal, Maclayton Paulyann I, Trachtenberg Barry H, Al-Mallah Mouaz,
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Gender Differences in Rates of Arrhythmias, Cardiac Implantable Electronic Devices, and Diagnostic Modalities Among Sarcoidosis Patients.
Cureus2020 Apr;12(4):e7667. doi: 10.7759/cureus.7667.
Durugu Satya, Gonuguntla Karthik, Patil Shivaraj, Rojulpote Chaitanya, Vyata Vishruth, Karambelkar Pranav, Narayanareddy Pranathi, Vuthaluru Kiranmayi, Bhattaru Abhijit,
Abstract
Introduction Sarcoidosis is a granulomatous disease with multiorgan involvement. Cardiac involvement may be asymptomatic or present clinically as heart failure, arrhythmias, or even sudden cardiac death. In this study, we compared gender differences in the prevalence of arrhythmias and associated outcomes in patients with sarcoidosis without established coronary artery disease. Methods The United States Nationwide Inpatient Sample was queried from 2010 to 2014 to identify patients with sarcoidosis using the International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code in patients >18 years. We excluded patients with a prior history of myocardial infarction, percutaneous coronary intervention, and coronary artery bypass graft. The chi-square test was used for statistical analysis. Results The sample consisted of 308,064 patients (mean age = 55.65 ± 11.28 years); they were mostly women (65.2%) and black (46.7%). In-hospital mortality in this cohort was 2.5%. The most common arrhythmia was atrial fibrillation (9.7%). The prevalence of ventricular fibrillation was 0.2%, ventricular tachycardia 2%, complete heart block 0.5%, and second-degree Mobitz type II (0.1%). Sudden cardiac death occurred in 0.7%. Rates of various cardiac devices implanted were: implantable cardiac defibrillator (ICD) (0.5%), cardiac resynchronization therapy-defibrillator (CRT-D) (0.2%), pacemaker (0.4%). Rates of endomyocardial biopsy (EMB), radionuclide imaging, and cardiac magnetic resonance imaging (MRI) were 0.2%, 0.3%, and 0.1%, respectively. Based on gender (male vs. female), the rates of arrhythmias, cardiac device implantation, and utilization of diagnostic modalities were: atrial fibrillation (41% vs 59%; p<0.001), ventricular fibrillation (50% vs 50%; p=0.983), ventricular tachycardia (55% vs 45%; p<0.001), complete heart block (48% vs 52%; p=0.3), second-degree Mobitz type II (37% vs 63%; p=0.706), sudden cardiac death (38% vs 62%; p<0.171), ICD (56% vs 44%; p<0.001), CRT-D (58% vs 42%; p=0.025), permanent pacemaker (40% vs 60%; p=0.066), EMB (55% vs 45%; p<0.001), radionuclide imaging (32% vs 68%; p=0.403), and cardiac MRI (41% vs 59%; p=0.396). In-hospital mortality was higher in females (64% vs 36%; p<0.001). Conclusion In our study, in-hospital death was more common in females. Females had higher rates of atrial fibrillation as compared to males, who were found to have a higher burden of ventricular tachycardia. Males had higher rates of ICD and CRT-D placement. Males also had EMB performed more commonly than females.
Copyright © 2020, Durugu et al.
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Serum angiotensin converting enzyme, Erythrocyte sedimentation rate and high sensitive-C reactive protein levels in diagnosis of cardiac sarcoidosis- where do we stand?
Indian Pacing Electrophysiol J2020 May;():. doi: S0972-6292(20)30059-0.
Bera Debabrata, Shanthi Naidu K, Kaur Saggu Daljeet, Yalagudri Sachin, Kishor Kadel Jugal, Sarkar Rakesh, Narasimhan C,
Abstract
BACKGROUND:
Cardiac sarcoidosis (CS) is increasingly being recognized in the last two decades. The diagnosis of CS depends on clustering of multiple symptoms, investigations and demonstration of a non-caseating granuloma on histopathology. Serum Angiotensin Converting Enzyme (SACE) level, one of the serological markers, is often elevated in systemic sarcoidosis. However, the yield of SACE level among patients with isolated or predominant CS is unclear. We conducted a retrospective study to assess the prevalence of elevated SACE level among patients with proven CS.
MATERIALS AND METHODS:
From our Granulomatous myocarditis (GM) registry, 45 biopsy proven CS patients were enrolled.
INCLUSION CRITERIA:
Clinical diagnosis of CS [HRS definition + Lymph Node biopsy/Endomyocardial biopsy (non-caseating granuloma)]. Exclusion criteria - Other causes of GM like cardiac tuberculosis (TB culture/AFB smear -positive) and patients taking medications affecting SACE level.
RESULTS:
Among 143?GM cases, 45 CS were analyzed. Mean age:42?±?11 years (Range 22-63 years, 19 females). With our laboratory reference of SACE (Normal range: 20-70 U/L), 3 out of 45 (6.7%) patients of CS had elevated SACE. In a comparative analysis we found, Erythrocyte Sedimentation Rate (ESR) and High sensitive-C Reactive Protein (Hs-CRP) are much more sensitive, although not specific for CS. Patients with pulmonary involvement more often had elevated SACE level.
CONCLUSION:
Serum ACE is elevated only in approximately 6.7% of patients with biopsy proven CS. Hence, it is insensitive serological tool for diagnosis of CS even in the active phase of the disease. In contrast, ESR and Hs-CRP emerges to be more sensitive markers of active CS.
Copyright © 2020 Indian Heart Rhythm Society. Production and hosting by Elsevier B.V. All rights reserved.
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Risk of Adverse Cardiovascular Events in Cardiac Sarcoidosis Independent of Left Ventricular Function.
Am J Cardiol2020 Apr;():. doi: S0002-9149(20)30394-5.
Rosenthal David G, Cheng Richard K, Petek Bradley J, Masri Sofia Carolina, Mikacenic Carmen, Raghu Ganesh, Patton Kristen K,
Abstract
This study investigated the association between left ventricular ejection fraction (LVEF) and the risk of ventricular arrhythmias (VA), heart transplantation, and death in cardiac sarcoidosis (CS). We identified 110 CS patients meeting 2014 Heart Rhythm Society (HRS) diagnostic criteria with baseline LVEF <35% (n?=?32) or ?35% (n?=?78). The primary end point was sustained VA or sudden cardiac death (SCD), and secondary end points included risk of heart transplantation, death, or a composite. Logistic regression determined risk factors for VA/SCD, and Cox proportional hazards regression analysis was performed for secondary end points. Receiver operating curve analysis determined the best discrimination point of LVEF for each end point; sensitivity analyses evaluated the effects of higher LVEF on each end point. Over a follow-up of 2.6 (range 1.0 to 5.8) years, 49 (44.5%) CS patients experienced VA/SCD, including 19 of 32 (59.4%) with LVEF <35%, and 30 of 78 (38.5%) with LVEF ?35%. After adjustment, LVEF <35% was not significantly associated with an increased risk of VA/SCD compared with LVEF ?35% (odds ratio 1.3, 95% confidence intervals 0.5 to 3.7). Although LVEF <35% was associated with an increased risk of heart transplantation and death (28.1% vs 12.8%, p?=?0.05), this was not significant after adjustment (hazard ratio 1.7, 95% confidence intervals 0.5 to 9.0, p?=?0.53). In conclusion, patients with CS experience high rates of VA, SCD, and heart transplantation, even when LVEF is mildly impaired or normal. Patients with LVEF <35% are at particularly elevated risk of VA/SCD. Our findings highlight the imperative to investigate arrhythmia risk in all patients with CS, even in the setting of an otherwise reassuring LVEF.
Copyright © 2020 Elsevier Inc. All rights reserved.
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Diagnostic accuracy of cardiac MRI, FDG-PET, and myocardial biopsy for the diagnosis of cardiac sarcoidosis: a protocol for a systematic review and meta-analysis.
Diagn Progn Res2020 ;4():5. doi: 10.1186/s41512-020-00073-4.
Roth Dominik, Kadoglou Nikolaos, Leeflang Mariska, Spijker Rene, Herkner Harald, Trivella Marialena,
Abstract
Background:
CS constitutes a rare but potentially underdiagnosed and fatal disease. Its diagnosis remains difficult owing to the infrequent and indistinguishable symptoms and the lack of formal diagnostic criteria dependent upon the diagnostic techniques used. Early diagnosis and treatment, however, may help to counter its poor prognosis.We aim to characterize and compare the diagnostic accuracy of cardiac MRI, FDG-PET and myocardial biopsy for the diagnosis of cardiac sarcoidosis and to advance and compare methods for complex diagnostic test accuracy reviews and meta-analysis.
Methods:
Following a systematic review on DTA studies on the aforementioned topic, a four-part approach to meta-analysis will be used: (1) direct comparison of index tests with clinical reference standard, (2) indirect comparison of index tests with clinical reference standard, (3) addition of an alternative test to that indirect comparison (4) and Bayesian meta-analysis using results of part 3 as informative prior for comparisons analogous to part 1 and 2.
Discussion:
The most widely recognized diagnostic algorithm for cardiac sarcoidosis is considered out of date, as it precedes the introduction of imaging techniques in diagnostic pathways. These novel imaging techniques, like CMR and FDG-PET scan, have emerged as promising diagnostic tools which may fill the current diagnostic gap. Thus, a systematic review and evaluation of CS diagnosis are much needed. Such an attempt is anticipated to alter the current diagnostic guidelines for CS by shedding more light on the role of sophisticated imaging techniques on prompt CS therapy and follow-up.
Trial registration:
PROSPERO, CRD42019047126.
© The Author(s) 2020.
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Arguable ICD placement in a sarcoidosis patient with extensive cardiac involvement.
Arch Med Sci2020 ;16(3):707-708. doi: 10.5114/aoms.2019.86708.
Jankowska Hanna, Dorniak Karolina, Hellmann Marcin, Dubaniewicz Anna, Dudziak Maria,
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A new electrocardiographic parameter associated with sudden cardiac death in pulmonary sarcoidosis.
Arch Med Sci2020 ;16(3):559-568. doi: 10.5114/aoms.2019.88393.
Emet Samim, Onur Seda T, Sokucu Sinem N, Aydin Senay, Dalar Levent, Cetinkaya Erdogan, Onur Imran,
Abstract
Introduction:
The interval from the peak to the end of the electrocardiographic T wave (T-T) may correspond to malignant ventricular arrhythmias. In this study we aimed to assess T-T variability and investigate the transmural dispersion of repolarisation in pulmonary sarcoidosis disease without proofed cardiac involvement.
Material and methods:
This was a retrospective case-control study that included patients who had a pathologic and radiologic diagnosis of sarcoidosis. All data of the patients' demographic features and electrocardiographs were analysed.
Results:
We enrolled 78 patients with sarcoidosis and 54 healthy volunteers as controls in our study. Men comprised 36% of the sarcoidosis group and 27% of controls. The mean age in the sarcoidosis and control group was 45.4 ±8.7 years (range: 23-58 years) and 44.6 ±11.9 years (range: 21-73 years), respectively. There was no significant difference between the groups for age or sex ( = 0.654, = 0.246, respectively). There was a significant increase in T-T results in all precordial leads in the sarcoidosis group compared with the control group ( < 0.05).
Conclusions:
Pulmonary sarcoidosis is suspected to have cardiac involvement; therefore, we need to develop new approaches. We present strong evidence that T-T intervals were increased in patients with pulmonary sarcoidosis, which suggests that there may be a link between sarcoidosis and ventricular arrhythmias without proofed cardiac involvement.
Copyright: © 2019 Termedia & Banach.
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[Sarcoidosis with Cardiac Involvement and Monoclonal Gammopathy].
Kardiologiia2020 Mar;60(4):151-156. doi: 10.18087/cardio.2020.4.n712.
Voronkova O O, Tsvetkova O A, Avdeev S N, Rogova E F, Abdullaeva G B,
Abstract
The article presented a clinical case of 8-year follow-up of a patient with a relatively beneficial course of pulmonary sarcoidosis and intrathoracic lymph nodes and with gradually progressing myocardial injury. A specific feature of the case was that the patient had monoclonal gammopathy, which is extremely rarely reported. Morphological signs of paraproteinemic hemoblastosis and amyloidosis were not observed. The article considered a whole complex of studies, which is required for diagnosing cardiac sarcoidosis in a specific patient and addressed the issue of monoclonal gammopathy origination.
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Cardiovascular magnetic resonance native T and T quantitative values for cardiomyopathies and heart transplantations: a systematic review and meta-analysis.
J Cardiovasc Magn Reson2020 May;22(1):34. doi: 10.1186/s12968-020-00627-x.
Snel G J H, van den Boomen M, Hernandez L M, Nguyen C T, Sosnovik D E, Velthuis B K, Slart R H J A, Borra R J H, Prakken N H J,
Abstract
BACKGROUND:
The clinical application of cardiovascular magnetic resonance (CMR) T and T mapping is currently limited as ranges for healthy and cardiac diseases are poorly defined. In this meta-analysis we aimed to determine the weighted mean of T and T mapping values in patients with myocardial infarction (MI), heart transplantation, non-ischemic cardiomyopathies (NICM) and hypertension, and the standardized mean difference (SMD) of each population with healthy controls. Additionally, the variation of mapping outcomes between studies was investigated.
METHODS:
The PRISMA guidelines were followed after literature searches on PubMed and Embase. Studies reporting CMR T or T values measured in patients were included. The SMD was calculated using a random effects model and a meta-regression analysis was performed for populations with sufficient published data.
RESULTS:
One hundred fifty-four studies, including 13,804 patient and 4392 control measurements, were included. T values were higher in patients with MI, heart transplantation, sarcoidosis, systemic lupus erythematosus, amyloidosis, hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and myocarditis (SMD of 2.17, 1.05, 0.87, 1.39, 1.62, 1.95, 1.90 and 1.33, respectively, P?
CONCLUSIONS:
The clinical utility of T and T mapping to distinguish affected myocardium in patients with cardiomyopathies or heart transplantation from healthy myocardium seemed to be confirmed based on this meta-analysis. Nevertheless, variation of mapping values between studies complicates comparison with external values and therefore require local healthy reference values to clinically interpret quantitative values. Furthermore, disease differentiation seems limited, since changes in T and T values of most cardiomyopathies are similar.
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Opportunities to diagnose fibrotic lung diseases in routine care: A primary care cohort study.
Respirology2020 May;():. doi: 10.1111/resp.13836.
Jones Mark G, Hillyar Christopher R T, Nibber Anjan, Chisholm Alison, Wilson Andrew, Maher Toby M, Kaplan Alan, Price David, Walsh Simon, Richeldi Luca, ,
Abstract
BACKGROUND AND OBJECTIVE:
Temporal trends of healthcare use in the period before a diagnosis of pulmonary fibrosis are poorly understood. We investigated trends in respiratory symptoms and LR HRU in the 10?years prior to diagnosis.
METHODS:
We analysed a primary care clinical cohort database (UK OPCRD) and assessed patients aged ?40?years who had an electronically coded diagnosis of pulmonary fibrosis between 2005 and 2015 and a minimum 2?years of continuous medical records prior to diagnosis. Exclusion criteria consisted of electronic codes for recognized causes of pulmonary fibrosis such as CTD, sarcoidosis or EAA.
RESULTS:
Data for 2223 patients were assessed. Over the 10?years prior to diagnosis of pulmonary fibrosis, there was a progressive increase in HRU across multiple LR-related domains. Five years before diagnosis, 18% of patients had multiple healthcare contacts for LR complaints; this increased to 79% in the year before diagnosis, with 38% of patients having five or more healthcare contacts.
CONCLUSION:
There are opportunities to diagnose pulmonary fibrosis at an earlier stage; research into case-finding algorithms and strategies to educate primary care physicians is required.
© 2020 Asian Pacific Society of Respirology.
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Concurrent cardiac sarcoidosis and obstructive sleep apnea presenting as arrhythmias.
BMC Pulm Med2020 May;20(1):132. doi: 10.1186/s12890-020-1163-5.
Malli Foteini, Bardaka Fotini, Gourgoulianis Konstantinos I, Daniil Zoe,
Abstract
BACKGROUND:
Cardiac involvement is a rare and potentially fatal presentation of sarcoidosis. Obstructive sleep apnea may complicate sarcoidosis.
CASE PRESENTATION:
We report a case of a sarcoidosis patient with cardiac involvement presenting with ventricular arrhythmias. Besides medical and invasive measures of therapy, the patient failed to respond fully. The patient was subjected to overnight polysomnography and diagnosed with concurrent obstructive sleep apnea syndrome. Following continuous positive airway pressure therapy, we observed a significant improvement of ventricular arrhythmias while methylprednisolone was further tapered.
CONCLUSIONS:
To our knowledge, this is the first report of cardiac sarcoidosis further implicated by OSAHS and presenting as ventricular arrhythmias that underlies the need for extensive testing in cardiac sarcoidosis in patients not responding to immunosuppressive therapy.
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Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct from Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy.
Circulation2020 May;():. doi: 10.1161/CIRCULATIONAHA.119.044934.
Smith Eric D, Lakdawala Neal K, Papoutsidakis Nikolaos, Aubert Gregory, Mazzanti Andrea, McCanta Anthony C, Agarwal Prachi P, Arscott Patricia, Dellefave-Castillo Lisa M, Vorovich Esther E, Nutakki Kavitha, Wilsbacher Lisa D, Priori Silvia G, Jacoby Daniel L, McNally Elizabeth M, Helms Adam S,
Abstract
Mutations in desmoplakin (), the primary force transducer between cardiac desmosomes and intermediate filaments, cause an arrhythmogenic form of cardiomyopathy that has been variably associated with arrhythmogenic right ventricular cardiomyopathy (ARVC). Clinical correlates of cardiomyopathy have been limited to small case series. Clinical and genetic data were collected on 107 patients with pathogenic mutations and 81 patients with pathogenic mutations as a comparison cohort. A composite outcome of severe ventricular arrhythmia was assessed. and cohorts included similar proportions of probands (41% vs. 42%) and patients with truncating mutations (98% vs. 100%). Left ventricular (LV) predominant cardiomyopathy was exclusively present among patients (55% vs. 0% for , p<0.001), whereas right ventricular (RV) cardiomyopathy was present in only 14% of patients vs. 40% for (p<0.001). ARVC diagnostic criteria had poor sensitivity for cardiomyopathy. LV late gadolinium enhancement (LGE) was present in a primarily subepicardial distribution in 40% of patients (23/57 with MRIs). LV LGE occurred with normal LV systolic function in 35% (8/23) of patients. Episodes of acute myocardial injury (chest pain with troponin elevation and normal coronary angiography) occurred in 15% of patients and were strongly associated with LV LGE (90%), even in cases of acute myocardial injury with normal ventricular function (4/5, 80% with LGE). In 4 cases with F-fluorodeoxyglucose positron emission tomography scans, acute LV myocardial injury was associated with myocardial inflammation misdiagnosed initially as cardiac sarcoidosis or myocarditis. LVEF <55% was strongly associated with severe ventricular arrhythmias for cases (p<0.001, sensitivity 85%, specificity 53%). RVEF <45% was associated with severe arrhythmias for cases (p<0.001) but was poorly associated for cases (p=0.8). Frequent PVCs were common among patients with severe arrhythmias for both (80%) and (91%) groups (p=NS). cardiomyopathy is a distinct form of arrhythmogenic cardiomyopathy characterized by episodic myocardial injury, left ventricular fibrosis that precedes systolic dysfunction, and a high incidence of ventricular arrhythmias. A genotype specific approach for diagnosis and risk stratification should be used.
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Predicting pulmonary hypertension in sarcoidosis; value of PH probability on echocardiography.
Int J Cardiovasc Imaging2020 Apr;():. doi: 10.1007/s10554-020-01859-9.
Huitema M P, Bakker A L M, Mager J J, Snijder R J, Rensing B J W M, Swaans M J, Grutters J C, Post M C,
Abstract
Pulmonary hypertension (PH) is a well-recognised complication of sarcoidosis. Non-invasive diagnosis is challenging due to limited accuracy of echocardiography in interstitial lung disease. This study evaluates the value of echocardiographic PH probability for diagnosing PH in pulmonary sarcoidosis. All consecutive patients between August 2015 and November 2018 were prospectively screened for PH, and classified as low, intermediate or high PH probability. Patients with intermediate or high PH probability were referred for right heart catheterisation. PH was defined as a mean pulmonary artery pressure of???25 mm Hg. Additional data on pulmonary function and chest-CT was collected. Of all 479 patients, PH was present in 17 and absent in 19 patients. Six patients refused right heart catheterisation. PH was present in 33% and 75% of patients with intermediate and high PH probability respectively (n?=?36). TRV max was measurable in 46% of all patients. Measurability did not correlate with FVC% predicted or presence of significant fibrosis. In intermediate and high PH probability, TRV max??3.4 confirmed PH in all patients. If TRV max was absent or in between 2.9 and 3.4, secondary echocardiographic signs were not able to improve the diagnostic accuracy. PH is unlikely in patients with a TRV max??3.4 m/s. Discrimination is challenging if the TRV max is between 2.9-3.4 m/s or absent. Additional secondary signs do not improve discrimination. Decision making for further investigations should be made by an expert team.
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Idiopathic giant cell myocarditis or cardiac sarcoidosis? A retrospective audit of a nationwide case series.
ESC Heart Fail2020 Apr;():. doi: 10.1002/ehf2.12725.
Ekström Kaj, Räisänen-Sokolowski Anne, Lehtonen Jukka, Nordenswan Hanna-Kaisa, Mäyränpää Mikko I, Kupari Markku,
Abstract
AIMS:
Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) are inflammatory cardiomyopathies sharing histopathological and clinical features. Their differentiation is difficult and susceptible of confusion and apparent mistakes. The possibility that they represent different phenotypes of a single disease has been debated.
METHODS AND RESULTS:
We made a retrospective audit of 73 cases of GCM diagnosed in Finland since the late 1980s. All available histological material was reanalyzed as were other examinations pertinent to the distinction between GCM and CS. Finding granulomas in or outside the heart was considered diagnostic of CS and exclusive of GCM. Altogether 45 of the 73 cases of GCM (62%) were reclassified as CS. In all except one case, this was based on finding sarcoid granulomas that either had been originally missed (n = 29) or misinterpreted (n = 11) or were found in additional posttransplant myocardial specimens (n = 3) or samples of extracardiac tissue (n = 1) accrued over the disease course. Supporting the reclassification, patients relocated to the CS group had less heart failure at presentation (prevalence 20% vs. 46%, P = 0.017) and better 1 year transplant-free survival (82% vs. 45%, P = 0.011) than patients considered to represent true GCM.
CONCLUSIONS:
Recognizing granulomas in or outside the heart remains a challenge for the pathologist. Given that CS and GCM are considered distinct diseases and granulomas exclusive of GCM, many cases of GCM, if thoroughly scrutinized, may need reclassification as CS. However, whether CS and GCM are truly different entities or parts of a one-disease continuum has not yet been conclusively settled.
© 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.
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Review: Contrast-enhanced magnetic resonance in the diagnosis and management of cardiac sarcoidosis.
Prog Cardiovasc Dis2020 Apr;():. doi: S0033-0620(20)30065-7.
Smedema Jan-Peter, Ainslie Gillian, Crijns Harry J G M,
Abstract
Sarcoidosis is a relatively rare inflammatory condition which potentially carries high morbidity and substantial mortality. Due to the fact that it does not subject patients to ionizing radiation, has high temporal, spatial and contrast resolutions, cardiovascular magnetic resonance imaging (CMR) has become an important diagnostic and prognostic modality in the evaluation for cardiac involvement in this condition. This review provides relevant clinical and pathophysiological background on cardiac sarcoidosis, whilst detailing the role of CMR imaging in the diagnosis, and management of this condition.
Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.
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The role of 18FDG PET/CT in the assessment of endocarditis, myocarditis and pericarditis.
Curr Radiopharm2020 Apr;():. doi: 10.2174/1874471013666200422102406.
Giorgetti Assuero, Genovesi Dario, Emdin Michele,
Abstract
BACKGROUND:
Endocarditis, myocarditis and pericarditis are a heterogeneous group of phenotypic syndromes where the culprit area of inflammation is the heart. Inflammation may be determined by an infective agent, toxins, drugs or an activated immune system. Clinical manifestations can be subtle and diagnosis remains a challenge for cardiologists, requires high level of suspicion and advanced multimodal cardiac imaging to avoid life-threatening consequences.
OBJECTIVE:
The purpose of this review is to report the recent advances of PET/CT imaging with 18FDG in helping the management of patients affected by inflammatory heart disease.
METHODS:
Two independent reviewers searched in PubMed articles published before or on June 2019 and final decisions on the inclusion of references were done in consensus with a third reviewer. At the end of the selection process were included 23/206 articles on "cardiac inflammation"; 26/235 articles on "endocarditis", "prosthetic heart valve", "pacemaker", "implantable cardiac device"; 7/103 articles on "myocarditis"; 13/330 articles on sarcoidosis" and 2/19 articles on "pericarditis".
CONCLUSION:
Compared with conventional methods, molecular imaging has the advantage to noninvasively and directly trace the inflammatory process, and to early identify the presence and the extent of intra-cardiac and extra-cardiac involvement, to enable quantification of disease activity, guide therapeutic interventions, and monitor treatment success.
Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.
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Prevalence of atrial FDG uptake and association with atrial arrhythmias in patients with cardiac sarcoidosis.
Int J Cardiol2020 Apr;():. doi: S0167-5273(20)30158-3.
Yodogawa Kenji, Fukushima Yoshimitsu, Ando Takahiro, Iwasaki Yu-Ki, Akiyama Kazuyoshi, Kumita Shin-Ichiro, Azuma Arata, Seino Yoshihiko, Shimizu Wataru,
Abstract
BACKGROUND:
There is increasing evidence that a proportion of patients with cardiac sarcoidosis (CS) have atrial arrhythmias (AA). Although 18F-fluorodeoxy-glucose (FDG) uptake in the ventricle on positron emission tomography/computed tomography (PET/CT) is well studied, FDG uptake in the atrium has not been elucidated in detail.
OBJECTIVES:
To evaluate FDG uptake in the atrium and its relationship with AA in patients with CS.
METHODS:
We retrospectively investigated 62 CS patients. All patients underwent echocardiography and PET/CT. Serum angiotensin converting enzyme (ACE) and soluble IL-2 receptor (sIL-2R) levels, plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) concentrations were also evaluated. ECG, Holter monitoring and device interrogations were used to detect AA.
RESULTS:
Of the studied population, 25 patients (40.3%) had AA, of which 2 patients had atrial tachycardia (AT) and 23 patients had atrial fibrillation (AF). Eighteen patients with AA had atrial FDG uptake on PET/CT, whereas 14 patients without AA had atrial FDG uptake (72.0% vs 37.8%, P = 0.017). Multivariate analysis revealed a significant association between AA and age (odds ratio [OR]: 1.15; 95% confidence interval [CI]: 1.01-1.31, P = 0.040), atrial FDG uptake (odds ratio [OR]: 7.23; 95% confidence interval [CI]: 1.91-27.36, P = 0.004), and left atrial diameter (OR: 1.08; 95% CI: 1.01-1.16, P = 0.027). Meanwhile, gender, serum ACE and BNP levels, and left ventricular ejection fraction were not associated with AA.
CONCLUSIONS:
Atrial FDG uptake was common in patients with CS and strongly associated with AA.
Copyright © 2020 Elsevier B.V. All rights reserved.
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Underdiagnosis of VT due to cycle length variation among cardiac sarcoidosis patients having ICD: Problem with stability discriminator.
Pacing Clin Electrophysiol2020 Apr;():. doi: 10.1111/pace.13923.
Bera Debabrata, Pavri Behzad B, Saggu Daljeet Kaur, Devidutta Soumen, Yalagudri Sachin, Subramanian Muthiah, Sridevi Chennapragada, Narasimhan Calambur,
Abstract
BACKGROUND:
Implantable cardioverter defibrillator (ICD) is recommended for patients with ventricular tachycardia (VT) due to cardiac sarcoidosis (CS). Programming supraventricular tachycardia (SVT) discriminators (onset, stability, and morphology/template match) is generally recommended to minimize inappropriate therapies. However, VT in patients with CS is known to show cycle length variability (CLV) and pleomorphism.
OBJECTIVE:
To determine whether the stability criterion, designed to prevent inappropriate therapy during atrial fibrillation with rapid ventricular rates, could potentially lead to incorrect classification of VT as SVT and inappropriately delay or inhibit ICD therapy.
METHODS:
Cases of biopsy-proven CS with VT were analyzed. For patients with implanted devices, all recorded electrograms of tachycardia episodes and ICD therapies were analyzed at last follow up.
RESULTS:
A total of 142 patients were included (mean age 38 years, 87 males). One hundred and three of 142 patients had implanted devices (ICD or CRT-D). Thirty eight of 103 (36.9%) patients received appropriate ICD therapies over 3 ± 2.2 years follow up. Four of 38 (10.5%) of patients experienced delayed-detection or underdetection of VT related to CLV, resulting in VT counters being repeatedly "reset" (classified as "unstable" rhythms). Retrospective analysis of other VT episodes in 70 of 103 (68%) patients revealed that 25 of 80 (31.3%) episodes had > 50 ms cycle length oscillations.
CONCLUSION:
Among CS patients with VT, CLV is a common occurrence seen in two-thirds of VT episodes. Routine programming of the stability criterion may result in underdetection of VT in a subset of such patients. We recommend that the stability criterion should be programmed "OFF" for patients with CS, unless the patient has documented atrial fibrillation.
© 2020 Wiley Periodicals, Inc.
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A Rare and Fatal Case of Hemophagocytic Lymphohistiocytosis Associated with Sarcoidosis.
Am J Case Rep2020 Apr;21():e921306. doi: 10.12659/AJCR.921306.
Yousif Patrick A, Moshrefi Hameadreza R, Mohamed Mohamed A, Meysami Alireza,
Abstract
BACKGROUND Sarcoidosis is a systemic inflammatory disorder characterized by a classic pathologic feature of non-caseating granulomas involving any organ system. Hemophagocytic lymphohistiocytosis (HLH) is a catastrophic cytokine surge characterized by dysregulation of the macrophage response, which can be rapidly fatal. Recognition of HLH has been increasing over the past decade. HLH can present with features of sepsis that can make the diagnosis challenging and requires high clinical suspicion. CASE REPORT We report a case of a 48-year-old African American male with a past medical history of sarcoidosis infiltrating the lymph nodes, liver, and bone marrow with initial presentation of abdominal pain, nausea, vomiting, and weight loss of 100 pounds over 8 months. Sepsis was suspected, but the patient clinically deteriorated with vancomycin and cefepime. Fevers, bone marrow biopsy, anemia, thrombocytopenia, elevated ferritin, and elevated soluble receptor interleukin 2 confirmed HLH. The patient was treated with etoposide and dexamethasone with poor response and died from cardiac arrest. CONCLUSIONS Sarcoidosis associated with HLH is an extremely rare phenomenon with only 10 cases reported in the literature. Early clinical suspicion can be challenging as this condition is a sepsis-mimicker. To reduce mortality, prompt initiation of therapy is a key determinant in patients who are clinically deteriorating despite treatment for sepsis.
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