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Pubblicazioni recenti - cardiac sarcoidosis
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Excellent suppression of physiological myocardial FDG activity in patients with cardiac sarcoidosis.
J Med Imaging Radiat Oncol2020 Oct;():. doi: 10.1111/1754-9485.13121.
Sankaran Shyam S, Kyprianou Katerina, Cherk Martin H, Nadebaum David P, Beech Paul A, Khor Robert, Zimmet Hendrik, Hare James L, Larby Annabel, Yap Kenneth Sk, Barber Thomas W,
Abstract
INTRODUCTION:
Suppression of physiological myocardial FDG activity is vital in patients undergoing PET/CT for assessment of known or suspected cardiac sarcoidosis. This study aims to evaluate the efficacy of physiological myocardial FDG suppression following a protocol change to a 24-h high fat very low carbohydrate (HFVLC) diet and prolonged fast.
METHODS:
A retrospective review of patients undergoing FDG PET/CT for the evaluation of cardiac sarcoidosis was performed. Prior to June-2018, patients were prepared with a single very high-fat low carbohydrate meal followed by a 12-18 h fast (group 1). After June-2018, a protocol change was initiated with patients prepared with a HFVLC diet for 24-h followed by a 12-18 h fast (group 2). Focal myocardial activity was classified as positive, absent activity as negative and diffuse/focal on diffuse activity as indeterminate.
RESULTS:
A total of 94 FDG PET/CT scans were included with 46 scans in group 1 and 48 scans in group 2. Studies were classified as positive, negative or indeterminate in 25 (54%), 7 (15%) and 14 (30%) scans in group 1 and in 13 (27%), 33 (69%) and 2 (4%) scans in group 2, respectively. In scans classified as negative, myocardial FDG activity was less than mediastinal blood pool activity in 5/7 (71%) scans in group 1 and 33/33 (100%) scans in group 2.
CONCLUSION:
Excellent myocardial FDG suppression can be achieved using a 24-h HFVLC diet and prolonged fast, resulting in a very low indeterminate scan rate in patients with known or suspected cardiac sarcoidosis.
© 2020 The Royal Australian and New Zealand College of Radiologists.
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High-dose intravenous glucocorticoids are effective in the acute management of ventricular arrhythmias in cardiac sarcoidosis: A case series.
HeartRhythm Case Rep2020 Oct;6(10):706-710. doi: 10.1016/j.hrcr.2020.06.028.
Slivnick Jeremy A, Betz Jarrod, Kalbfleisch Steven, Crouser Elliott D, Kahwash Rami,
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Ventricular fibrillation as an initial manifestation of cardiac sarcoidosis.
Proc (Bayl Univ Med Cent)2020 Jul;33(4):655-657. doi: 10.1080/08998280.2020.1785814.
Latif Azka, Patel Apurva D, Kuniyoshi Jason, Kapoor Vikas, Aggarwal Gaurav, Khan Behram Ahmed, Koster Nancy,
Abstract
Cardiac sarcoidosis is a rare immunologically driven process seen in 2% to 5% of patients with systemic sarcoidosis. We present a 31-year-old woman who presented after a ventricular fibrillation cardiac arrest. A comprehensive diagnostic workup was unrevealing. Despite negative cardiac magnetic resonance imaging, positron emission tomography facilitated the diagnosis. This case illustrates both the limitations of the diagnostic workup of sarcoidosis and the usefulness of positron emission tomography in the early diagnosis of cardiac sarcoidosis.
Copyright © 2020 Baylor University Medical Center.
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Cardiac sarcoidosis: A multimodal approach to reach the diagnosis.
Int J Cardiol -
Treatment of newly diagnosed sarcoid-associated pulmonary hypertension with ambrisentan and tadalafil combination therapy.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(2):234-238. doi: 10.36141/svdld.v37i2.9343.
Abston Eric, Hon Stephanie, Lawrence Romy, Berman Jeffrey, Govender Praveen, Farber Harrison W,
Abstract
Sarcoid Associated Pulmonary Hypertension (SAPH) is a common complication of sarcoidosis and is associated with poor prognosis. SAPH can be due to multiple synergistic mechanisms and current therapeutic strategies treat systemic sarcoidosis and pulmonary hypertension separately. Several studies have been performed to develop an effective therapy for SAPH but have been met with mixed results. The AMBITION trial successfully treated incident patients with pulmonary arterial hypertension (PAH) with the upfront combination of ambrisentan and tadalafil; however combination therapy has not yet been studied in patients with SAPH. Here we report a cohort of patients with newly diagnosed SAPH who were treated with upfront combination therapy per the AMBITION study protocol. We report three subjects with newly diagnosed SAPH who were treated with combination ambrisentan and tadalafil. Baseline hemodynamics were compared with those from surveillance right heart catheterization while on therapy. Mean follow up period was 17 months. Each subject demonstrated clinical and hemodynamic improvement with combination therapy. This series is the first to evaluate upfront combination ambrisentan and tadalafil therapy for treatment of newly diagnosed SAPH. Despite the impressive clinical and hemodynamic improvement, the study is limited by its small size and retrospective nature. While these initial results are promising, further work is needed to fully evaluate this regimen for treatment of SAPH. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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Associations between interleukin 18 gene polymorphisms and susceptibility to vasculitis: A meta-analysis.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(2):203-211. doi: 10.36141/svdld.v37i2.9399.
Jung Jae Hyun, Jeong Han Saem, Choi Sung Jae, Song Gwan Gyu, Kim Jong-Ho, Lee Tae Hyub, Han Youngjin,
Abstract
Interleukin 18 (IL18), a pro-inflammatory cytokine, affects the development and progress of vasculitis. The production, expression, and function of this cytokine are affected by polymorphisms of promoter region of the gene. In this study, a meta-analysis of the associations between several polymorphisms and susceptibility to vasculitis was performed. Published literature from PubMed and Embase were retrieved. In total, nine studies comprising 1006 patients with vasculitis and 1499 controls combined, and the investigating the rs187238, rs194618, and rs360719 polymorphisms of the promoter region of the gene, were included in the meta-analysis. Pooled odds ratios (OR) and 95% confidence intervals (CI) were estimated with fixed-effects model or random-effects model. The recessive model of the rs194618 polymorphism was found to be significantly associated with a high susceptibility to vasculitis (OR = 1.54, 95% CI = 1.02-2.33, = 0.04), especially in the Mongoloid race, where the allele of rs194618 was associated with a low risk of the disease (OR = 0.77, 95% CI = 0.62-0.95, = 0.01). By contrast, the rs187238 and rs360719 polymorphisms were not associated with this inflammatory condition. This meta-analysis showed that some polymorphisms are associated with susceptibility to vasculitis. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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Long-term outcomes of epoprostenol therapy in sarcoid associated pulmonary hypertension.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(2):184-191. doi: 10.36141/svdld.v37i2.9150.
Abston Eric, Moll Matthew, Hon Stephanie, Govender Praveen, Berman Jeffrey, Farber Harrison,
Abstract
Sarcoidosis-Associated Pulmonary Hypertension (SAPH) is a common finding in patients with chronic sarcoidosis and is associated with increased mortality. The optimal treatment for SAPH is not known; however, therapies approved for Group 1 pulmonary hypertension have improved hemodynamics and functional status. Prostanoids, including epoprostenol, have been therapeutic in short-term studies of SAPH, but long-term efficacy is unknown. In this study, we evaluated the long-term effect of epoprostenol therapy in 12 patients with SAPH. Hemodynamic assessment after an average of 4.1 years of epoprostenol therapy demonstrated significant improvement in mean pulmonary arterial pressure, pulmonary vascular resistance, and cardiac output; furthermore, patients demonstrated improved NYHA functional class. To evaluate further the long-term effect of epoprostenol, we compared survival of SAPH patients to a cohort of hemodynamically matched patients from the same center treated with epoprostenol for Idiopathic Pulmonary Arterial Hypertension (IPAH). Interestingly, there was no difference in survival, despite the additional systemic disease burden of the SAPH subjects. Subgroup analysis by Scadding stage demonstrated that Scadding stages 1-3 had improved survival compared to Scadding stage 4. These observations suggest that epoprostenol is an effective long-term therapy for patients with SAPH; it improves hemodynamics, functional class, and provides survival similar to that seen in a hemodynamically-matched cohort of IPAH patients. Furthermore, we identify a subgroup of SAPH patients (nonfibrotic lung disease Scadding 1-3) who may derive significant benefit from prostanoid therapy. (.
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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Sarcoidosis and increased risk of comorbidities and mortality in sweden.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(2):104-135. doi: 10.36141/svdld.v37i2.9142.
Larsson Johanna, Graff Pål, Bryngelsson Ing-Liss, Vihlborg Per,
Abstract
Introduction:
Sarcoidosis is a systemic inflammatory disorder, with an unclear etiology, involving granuloma formation that in most cases affects the lungs and intrathoracic lymph nodes. Sarcoidosis occurs in an acute or chronic form, each with different clinical presentation and prognosis.
Methods:
Case-control study of comorbidity and mortality in people diagnosed with sarcoidosis (ICD10 code D86) from 2007 through 2016 in Sweden. Controls were matched to cases (2:1) based on age, sex and county at the time of diagnosis. Data was collected from the Swedish National Patient Register and The Cause of Death Register. All men and women aged 20-65 years old who were diagnosed with sarcoidosis (D86, ICD10) during the years of study were included, resulting 7828 cases and 15656 controls.
Results:
Patients with sarcoidosis had increased mortality compared to matched controls (hazard ratio 1.88; 95% CI 1.56 - 2.26) and the Swedish general population (standardized mortality ratios1.75; 95% CI 1.52 - 2.00). The sarcoid cases, compared to controls, also had a significantly greater number of inpatient visits within several different chapters of ICD10 e.g. cardiomyopathy, heart failure, pulmonary embolism and malignant neoplasm.
Conclusion:
Individuals with sarcoidosis are at higher risk of comorbidities and mortality than matched controls as well as the general population of Sweden. These findings are important knowledge for healthcare professionals who meet sarcoid patients, to encourage identification and treatment of comorbidities to reduce the risk of impaired quality of life and, eventually, premature death. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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Infection prevention in sarcoidosis: proposal for vaccination and prophylactic therapy.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(2):87-98. doi: 10.36141/svdld.v37i2.9599.
Syed Huzaefah, Ascoli Christian, Linssen Catharina Fm, Vagts Christen, Iden Thomas, Syed Aamer, Kron Jordana, Polly Kelly, Perkins David, Finn Patricia W, Novak Richard, Drent Marjolein, Baughman Robert, Sweiss Nadera J,
Abstract
Sarcoidosis is a systemic inflammatory disease characterized by granuloma formation in affected organs and caused by dysregulated immune response to an unknown antigen. Sarcoidosis patients receiving immunosuppressive medications are at increased risk of infection. Lymphopenia is also commonly seen among patient with sarcoidosis. In this review, risk of infections, including opportunistic infections, will be outlined. Recommendations for vaccinations and prophylactic therapy based on literature review will also be summarized. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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Safety of macitentan in sarcoidosis-associated pulmonary hypertension: a case-series.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(1):74-78. doi: 10.36141/svdld.v37i1.9292.
Mathijssen H, Huitema M P, Bakker A L M, Mager J J, Snijder R J, Grutters J C, Post M C,
Abstract
Background:
Pulmonary hypertension (PH) is a known complication of pulmonary sarcoidosis and is associated with higher morbidity and mortality. Currently, there are no approved PH-targeted therapies for sarcoidosis-associated pulmonary hypertension (SAPH). Macitentan is frequently used as treatment for pulmonary arterial hypertension, but no results are known in the SAPH population.
Objective:
We investigated the safety and effect of macitentan as treatment for SAPH.
Methods:
We retrospectively reviewed our patient database for all SAPH patients receiving macitentan as treatment, with a minimum follow-up of twelve months for monitoring safety. Safety outcomes included reported side-effects, hospitalisations and mortality. Furthermore, six-minutes walking distance, New York Heart Association functional class and NT-proBNP levels were collected.
Results:
Six cases (three men) with a median age of 64 years (range 52-74 years) were identified. During macitentan treatment, one patient experienced side effects and aborted therapy after five days of treatment and died 16 months later. Three patients were hospitalised during treatment for congestive heart failure. Four patients showed improvement of their functional class and three patients in exercise capacity after 12 months of therapy.
Conclusion:
Macitentan was well tolerated in five out of six cases with severe pulmonary sarcoidosis and PH. Functional capacity improved in four cases. Prospective controlled trials are warranted before therapeutic recommendations can be made. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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The effect of global longitudinal strain on ?mpaired six-minute walk test performance in patients with sarcoidosis.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(1):66-73. doi: 10.36141/svdld.v37i1.8802.
Kaptan Ozen Deniz, Mutlu Bulent, Kocakaya Derya, Turan Burak, Sert Sekerci Sena, Ceyhan Berrin, Kepez Alper, Erdogan Okan,
Abstract
Background:
Sarcoidosis is a multisystem and granulomatous disease associated with impaired functional capacity as a result of pulmonary and cardiac involvement. Factors adversely effecting functional capacity in patients with sarcoidosis have not been systematically assessed including myocardial strain imaging on echocardiography which enable to diagnose subclinical cardiac dysfunction. We aimed to evaluate the effect of left and right ventricular global longitudinal strain (GLS) on submaximal exercise capacity in patients with sarcoidosis who do not have clinically manifest cardiac involvement.
Methods:
Extracardiac biopsy proven 56 patients with sarcoidosis and 26 controls were included consecutively. Submaximal exercise capacity of the subjects was assessed with six-minute walk test (6 MWT). Pulmonary function tests and standard transthoracic and two-dimensional speckle tracking echocardiography were performed to the all subjects. Linear regression analysis was performed to find independent predictors of 6 MWT.
Results:
Fifty-six patients (18% male) with a mean age of 52.5 ± 10.7 years were included. Patients with sarcoidosis had low 6 MWT performance and higher New York Heart Association classes and NT-proBNP levels. There were no significant differences between controls and patients with sarcoidosis in parameters of pulmonary function test. Biventricular GLS levels and biatrial reservoir and conduit function values were lower and systolic pulmonary artery pressure (SPAP) was significantly higher in patients with sarcoidosis as compared with controls. Older age and higher SPAP were found as independent predictors of poor 6 MWT performance.
Conclusion:
Although biventricular GLS levels were lower in the patients with sarcoidosis, only age and SPAP elevations were independent predictors of the submaximal exercise capacity. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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The incidence, comorbidity and mortality of sarcoidosis in Korea, 2008-2015: a nationwide population-based study.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(1):24-26. doi: 10.36141/svdld.v37i1.7660.
Jeon Mi Hye, Kang Taeuk, Yoo Sang Hoon, Swan Heather S, Kim Hyun Jung, Ahn Hyeong Sik,
Abstract
Background:
Few national level, population-based studies are present on the epidemiology of sarcoidosis and it is unclear whether these patients have higher mortality than the general population. The objective of this study was to investigate the nationwide epidemiology, comorbidity and mortality in sarcoidosis in Korea.
Material and Methods:
For the period between 2008 to 2015, we used the national population-based database operated by Rare Intractable Disease registration program in which patients' diagnosis are based on uniform criteria. All sarcoidosis patients were identified and followed-up using the National Health Insurance database to determine their incidence, comorbidity, mortality, causes of death and standardised mortality ratio (SMR).
Results:
During the study period, we identified 3,259 new sarcoidosis patients. The average annual incidence was 0.81 per 100,000. The annual mortality rate was 9.26 per 1,000 person-years. The mortality rate were significantly higher than those of the general population (SMR 1.91, 95% confidence interval 1.62-2.25). The major comorbidities of sarcoidosis patients were the diseases of the respiratory system (17.64%), heart (5.43%), eyes (4.27%) and cancer (2.3%). Mortality was higher in patients with lung involvement. Of the 84 deaths identified in this study from 2008-2013, the most common cause of death was cancer (41.7%), followed by respiratory disease (13.1%), sarcoidosis (13.1%) and heart disease (8.3%).
Conclusions:
We reported a nationwide incidence of sarcoidosis as 0.81 per 100,000 in Korea. The mortality of sarcoidosis patients was higher compared to the general population and the major causes of death were cancer, respiratory disease and sarcoidosis. Sarcoidosis patients with comorbid diseases showed increased mortality. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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Factors associated with implantable cardioverter defibrillators appropriate therapy in cardiac sarcoidosis: a meta-analysis.
Sarcoidosis Vasc Diffuse Lung Dis2020 ;37(1):17-23. doi: 10.36141/svdld.v37i1.8271.
Azoulay Levi-Dan, Waintraub Xavier, Haroche Julien, Amoura Zahir, Cohen Aubart Fleur,
Abstract
Background:
Patients with cardiac sarcoidosis (CS) are at increased risk of atrioventricular blocks, ventricular arrhythmias, and sudden cardiac death. Objectives We aimed to investigate the characteristics associated with appropriate therapy in implantable cardiac defibrillator (ICD) -implanted CS patients.
Methods:
We performed a PubMed and Web of Science search for studies reporting patients with CS who underwent an ICD implantation. The primary criterion was an appropriate therapy.
Results:
We screened 705 studies, of which 5 were included in the final analysis. We conducted a meta-analysis including 464 patients (mean age 55 years, 282 males (60%)). The mean follow-up was 3.5 years. Among the 464 patients, 180 received an appropriate therapy (39%). Patients who received an appropriate therapy were younger (-3.33, 95% confidence interval (CI) -6.42 to -0.23, p=0.004), were more likely to be male (OR 2.06, 95% CI 1.37-3.09, p=0.0005), had a lower left ventricular ejection fraction (LVEF) (-10.5, 95% CI -18.23 to -2.78, p=0.008), had a higher rate of complete heart block (OR 2.19, 95% CI 1.20 to 3.99, p=0.01), and more frequently had ventricular pacing (OR 6.44 95% CI 2.57 to 16.16, p<0.0001).
Conclusions:
Appropriate ICD therapy during CS is associated with young age, male sex, low LVEF, history of complete heart block, and ventricular pacing. .
Copyright: © 2020 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.
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Clinical phenotypes of extrapulmonary sarcoidosis: an analysis of a French, multiethnic, multicenter cohort.
Eur Respir J2020 Oct;():. doi: 2001160.
Lhote Raphael, Annesi-Maesano Isabella, Nunes Hilario, Launay David, Borie Raphael, Sacré Karim, Schleinitz Nicolas, Hamidou Mohamed, Mahevas Matthieu, Devilliers Hervé, Bonniaud Philippe, Lhote François, Haroche Julien, Rufat Pierre, Amoura Zahir, Valeyre Dominique, Cohen Aubart Fleur,
Abstract
BACKGROUND:
Sarcoidosis is a rare disease of unknown cause with wide heterogeneity in clinical features and outcomes. We aimed to explore sarcoidosis phenotypes and their clinical relevance with particular attention to extrapulmonary subgroups.
PATIENTS AND METHODS:
The EpiSarc (Epidemiology of Sarcoidosis) study is a French retrospective multicenter study. Sarcoidosis patients were identified through national hospitalisation records using appropriate codes from 11 hospital centers between 2013 and 2016 according to a standardised protocol. Medical charts were reviewed. The phenotypes of sarcoidosis were defined using a hierarchical cluster analysis.
RESULTS:
A total of 1237 patients were included (562 men and 675 women). The mean age at sarcoidosis diagnosis was 43.5±13?years. Hierarchical cluster analysis identified five distinct phenotypes according to organ involvement and disease type and symptoms: 1) (n=180) , joint involvement and hilar lymph nodes; 2) (n=137) eye, neurological, digestive and kidney involvement; 3) (n=630) pulmonary involvement with fibrosis and heart involvement; 4) (n=41) and a high percentage of severe involvement; and 5) (n=249) hepatosplenic, peripheral lymph node and bone involvement. Phenotype 1 was associated with being European and female and with nonmanual work; phenotype 2 with being European; and phenotypes 3 and 5 with being non-European. The labor worker proportion was significantly lower in phenotype 5 than in the other phenotypes.
ANSWER TO THE QUESTION:
This multicenter study confirms the existence of distinct phenotypes of sarcoidosis, with a nonrandom distribution of organ involvement. These phenotypes differ according to gender, geographical origin and socioprofessional categories.
Copyright ©ERS 2020.
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Cardiac sarcoidosis mimicking definite arrhythmogenic right ventricular cardiomyopathy.
Heart Rhythm -
Design of a randomized controlled trial to evaluate effectiveness of methotrexate versus prednisone as first-line treatment for pulmonary sarcoidosis: the PREDMETH study.
BMC Pulm Med2020 Oct;20(1):271. doi: 10.1186/s12890-020-01290-9.
Kahlmann Vivienne, Janssen Bonás Montse, Moor Catharina C, van Moorsel Coline H M, Kool Mirjam, Kraaijvanger Raisa, Grutters Jan C, Overgaauw Mayka, Veltkamp Marcel, Wijsenbeek Marlies S, ,
Abstract
BACKGROUND:
Treatment of pulmonary sarcoidosis is recommended in case of significant symptoms, impaired or deteriorating lung function. Evidence-based treatment recommendations are limited and largely based on expert opinion. Prednisone is currently the first-choice therapy and leads to short-term improvement of lung function. Unfortunately, prednisone often has side-effects and may be associated with impaired quality of life. Methotrexate is presently considered second-line therapy, and appears to have fewer side-effects.
OBJECTIVE:
The primary objective of this trial is to investigate the effectiveness and tolerability of methotrexate as first-line therapy in patients with pulmonary sarcoidosis compared with prednisone. The primary endpoint of this study will be the change in hospital-measured Forced Vital Capacity (FVC) between baseline and 24?weeks. Secondary objectives are to gain more insights in response to therapy in individual patients by home spirometry and patient-reported outcomes. Blood biomarkers will be examined to find predictors of response to therapy, disease progression and chronicity, and to improve our understanding of the underlying disease mechanism.
METHODS/DESIGN:
In this prospective, randomized, non-blinded, multi-center, non-inferiority trial, we plan to randomize 138 treatment-naïve patients with pulmonary sarcoidosis who are about to start treatment. Patients will be randomized in a 1:1 ratio to receive either prednisone or methotrexate in a predefined schedule for 24?weeks, after which they will be followed up in regular care for up to 2?years. Regular hospital visits will include pulmonary function assessment, completion of patient-reported outcomes, and blood withdrawal. Additionally, patients will be asked to perform weekly home spirometry, and record symptoms and side-effects via a home monitoring application for 24?weeks.
DISCUSSION:
This study will be the first randomized controlled trial comparing first-line treatment of prednisone and methotrexate and provide valuable data on efficacy, safety, quality of life and biomarkers. If this study confirms the hypothesis that methotrexate is as effective as prednisone as first-line treatment for sarcoidosis but with fewer side-effects, this will lead to improvement in care and initiate a change in practice. Furthermore, insights into the immunological mechanisms underlying sarcoidosis pathology might reveal new therapeutic targets.
TRIAL REGISTRATION:
The study was registered on the 19th of March 2020 in the International Clinical Trial Registry, www.clinicaltrials.gov; ID NCT04314193 .
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Cardiac sarcoidosis: diagnosis and management.
Rev Cardiovasc Med2020 Sep;21(3):321-338. doi: 10.31083/j.rcm.2020.03.102.
Markatis Eleftherios, Afthinos Andreas, Antonakis Emmanouil, Papanikolaou Ilias C,
Abstract
Sarcoidosis is a chronic inflammatory disease of unknown etiology characterized by multi-organ involvement. End-organ disease consists of granulomatous inflammation, which if left untreated or not resolved spontaneously, leads to permanent fibrosis and end-organ dysfunction. Cardiac involvement and fibrosis in sarcoidosis occur in 5-10% of cases and is becoming increasingly diagnosed. This is due to increased clinical awareness among clinicians and new diagnostic modalities, since magnetic resonance imaging and positron-emission tomography are emerging as "gold standard" tools replacing endomyocardial biopsy. Despite this progress, isolated cardiac sarcoidosis is difficult to differentiate from other causes of arrhythmogenic cardiomyopathy. Cardiac fibrosis leads to congestive heart failure, arrhythmias and sudden cardiac death. Immunosuppressives (mostly corticosteroids) are used for the treatment of cardiac sarcoidosis. Implantable devices like a cardioverter-defibrillator may be warranted in order to prevent sudden cardiac death. In this article current trends in the pathophysiology, diagnosis and management of cardiac sarcoidosis will be reviewed focusing on published research and latest guidelines. Lastly, a management algorithm is proposed.
© 2020 Markatis et al. Published by IMR press.
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Association of septal late gadolinium enhancement on cardiac magnetic resonance with ventricular tachycardia ablation targets in nonischemic cardiomyopathy.
J Cardiovasc Electrophysiol2020 Oct;():. doi: 10.1111/jce.14777.
Kuo Ling, Liang Jackson J, Han Yuchi, Frankel David S, Santangeli Pasquale, Callans David J, Zado Erica S, Marchlinski Francis E, Desjardins Benoit, Nazarian Saman,
Abstract
BACKGROUND:
Ablation of septal substrate-associated ventricular tachycardia (VT) in patients with nonischemic cardiomyopathy (NICM) is challenging. We sought to standardize the characterization of septal substrates on late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) and to examine the association of that substrate with VT exit and isthmus sites on invasive mapping.
METHODS:
LGE-CMR was performed before electroanatomic mapping and ablation for VT in 20 NICM patients. LGE extent and distribution were quantified using myocardial signal-intensity Z scores (SI-Z). The SI-Z thresholds correlating to previously validated voltage thresholds, for abnormal tissue and dense scar were defined.
RESULTS:
Bipolar and unipolar (electrogram) voltage amplitude measurements from the LV and RV were negatively associated with SI-Z from LGE-CMR imaging (p?
CONCLUSIONS:
Critical sites of septal VT in NICM patients are predominantly in the CMR defined dense scar when using standardized signal-intensity thresholds.
© 2020 Wiley Periodicals LLC.
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Quantitative clinical nuclear cardiology, part 2: Evolving/emerging applications.
J Nucl Cardiol2020 Oct;():. doi: 10.1007/s12350-020-02337-4.
Slomka Piotr J, Moody Jonathan B, Miller Robert J H, Renaud Jennifer M, Ficaro Edward P, Garcia Ernest V,
Abstract
Quantitative analysis has been applied extensively to image processing and interpretation in nuclear cardiology to improve disease diagnosis and risk stratification. This is Part 2 of a two-part continuing medical education article, which will review the potential clinical role for emerging quantitative analysis tools. The article will describe advanced methods for quantifying dyssynchrony, ventricular function and perfusion, and hybrid imaging analysis. This article discusses evolving methods to measure myocardial blood flow with positron emission tomography and single-photon emission computed tomography. Novel quantitative assessments of myocardial viability, microcalcification and in patients with cardiac sarcoidosis and cardiac amyloidosis will also be described. Lastly, we will review the potential role for artificial intelligence to improve image analysis, disease diagnosis, and risk prediction. The potential clinical role for all these novel techniques will be highlighted as well as methods to optimize their implementation.
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Quantitative Clinical Nuclear Cardiology, Part 2: Evolving/Emerging Applications.
J Nucl Med2020 Oct;():. doi: jnumed.120.242537.
Slomka Piotr J, Moody Jonathan B, Miller Robert J H, Renaud Jennifer, Ficaro Edward P, Garcia Ernest V,
Abstract
Quantitative analysis has been applied extensively to image processing and interpretation in nuclear cardiology in order to improve disease diagnosis and risk stratification. This is Part 2 of a two-part continuing medical education article, which will review the potential clinical role for emerging quantitative analysis tools. The article will describe advanced methods for quantifying dyssynchrony, ventricular function and perfusion, and hybrid imaging analysis. This article discusses evolving methods to measure myocardial blood flow with positron emission tomography and single photon emission computed tomography. Novel quantitative assessments of myocardial viability, microcalcification and in patients with cardiac sarcoidosis and cardiac amyloidosis will also be described. Lastly, we will review the potential role for artificial intelligence to improve image analysis, disease diagnosis, and risk prediction. The potential clinical role for all these novel techniques will be highlighted as well as methods to optimize their implementation.
Copyright © 2020 by the Society of Nuclear Medicine and Molecular Imaging, Inc.
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