Pubblicazioni recenti - cardiac sarcoidosis
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Cardiac Sarcoidosis-Diagnostic and Therapeutic Challenges.
J Clin Med2024 Mar;13(6):. doi: 1694.
Korthals Dennis, Bietenbeck Michael, Könemann Hilke, Doldi Florian, Ventura David, Schäfers Michael, Mohr Michael, Wolfes Julian, Wegner Felix, Yilmaz Ali, Eckardt Lars,
Abstract
Sarcoidosis is a multisystem disorder of unknown etiology. The leading hypothesis involves an antigen-triggered dysregulated T-cell-driven immunologic response leading to non-necrotic granulomas. In cardiac sarcoidosis (CS), the inflammatory response can lead to fibrosis, culminating in clinical manifestations such as atrioventricular block and ventricular arrhythmias. Cardiac manifestations frequently present as first and isolated signs or may appear in conjunction with extracardiac manifestations. The incidence of sudden cardiac death (SCD) is high. Diagnosis remains a challenge. For a definite diagnosis, endomyocardial biopsy (EMB) is suggested. In clinical practice, compatible findings in advanced imaging using cardiovascular magnetic resonance (CMR) and/or positron emission tomography (PET) in combination with extracardiac histological proof is considered sufficient. Management revolves around the control of myocardial inflammation by employing immunosuppression. However, data regarding efficacy are merely based on observational evidence. Prevention of SCD is of particular importance and several guidelines provide recommendations regarding device therapy. In patients with manifest CS, outcome data indicate a 5-year survival of around 90% and a 10-year survival in the range of 80%. Data for patients with silent CS are conflicting; some studies suggest an overall benign course of disease while others reported contrasting observations. Future research challenges involve better understanding of the immunologic pathogenesis of the disease for a targeted therapy, improving imaging to aid early diagnosis, assessing the need for screening of asymptomatic patients and randomized trials.
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Genetic Cardiomyopathies Misdiagnosed as Cardiac Sarcoidosis.
JACC Clin Electrophysiol2024 Mar;10(3):583-584. doi: 10.1016/j.jacep.2023.12.010.
Subramanian Muthiah, Ravikanth V V, Saggu Daljeet, Yalagudri Sachin, Narasimhan Calambur,
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Comparison of organ involvement clusters in Black and White American sarcoidosis patients from a prospectively collected patient registry.
Respir Med2024 Mar;():107605. doi: 10.1016/j.rmed.2024.107605.
Harper Logan J, Tauquir Anoosha, Huang Shuaiqi, Wang Xiaofeng, Schupp Jonas C, Baughman Robert, Culver Daniel A,
Abstract
BACKGROUND:
Due to the heterogeneity of sarcoidosis, there is a need to define clinical phenotypes to allow for tailoring of clinical care and identification of more homogenous populations to facilitate research.
METHODS:
We utilized data from a prospectively collected registry of sarcoidosis patients seen at a single quaternary referral center between January 2019 and February 2021. We used multiple correspondence analysis (MCA) and k-means clustering to investigate if the clusters previously identified in the GenPhenReSa study were reproducible in a US population. We also investigated if these clusters were stable when the population was stratified by race.
RESULTS:
We replicated 3 of the 5 clusters seen in the GenPhenReSa study in our cohort. We likewise identified similar clusters between White and Black patients with sarcoidosis. Differences in organ manifestations associations between White and Black patients were seen primarily in relation to cardiac, neurologic, and ocular involvement.
CONCLUSIONS:
The organ clusters of liver-spleen, isolated pulmonary, and musculoskeletal-skin were reproducible in a US cohort, and in both Black and White patients.
Copyright © 2024. Published by Elsevier Ltd.
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Trends and Disparities Around Cardiovascular Mortality in Sarcoidosis: Does Big Data Have the Answers?
J Am Heart Assoc2024 Mar;():e034073. doi: 10.1161/JAHA.124.034073.
Ahmed Raheel, Sharma Rakesh, Chahal C Anwar A,
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Trends and Disparities in Cardiovascular Death in Sarcoidosis: A Population-Based Retrospective Study in the United States From 1999 to 2020.
J Am Heart Assoc2024 Mar;():e031484. doi: 10.1161/JAHA.123.031484.
Tan Min Choon, Yeo Yong Hao, Mirza Noreen, San Boon Jian, Tan Jian Liang, Lee Justin Z, Mazzarelli Joanne K, Russo Andrea M,
Abstract
BACKGROUND:
Despite significant cardiac involvement in sarcoidosis, real-world data on death due to cardiovascular disease among patients with sarcoidosis is not well established.
METHODS AND RESULTS:
We queried the Centers for Disease Control and Prevention's Wide-Ranging Online Data for Epidemiologic Research database for data on patients with sarcoidosis aged ?25?years from 1999 to 2020. Diseases of the circulatory system except ischemic heart disease were listed as the underlying cause of death, and sarcoidosis was stated as a contributing cause of death. We calculated age-adjusted mortality rate (AAMR) per 1 million individuals and determined the trends over time by estimating the annual percentage change using the Joinpoint Regression Program. Subgroup analyses were performed on the basis of demographic and geographic factors. In the 22-year study period, 3301 cardiovascular deaths with comorbid sarcoidosis were identified. The AAMR from cardiovascular deaths with comorbid sarcoidosis increased from 0.53 (95% CI, 0.43-0.65) per 1 million individuals in 1999 to 0.87 (95% CI, 0.75-0.98) per 1?million individuals in 2020. Overall, women recorded a higher AAMR compared with men (0.77 [95% CI, 0.74-0.81] versus 0.58 [95% CI, 0.55-0.62]). People with Black ancestry had higher AAMR than people with White ancestry (3.23 [95% CI, 3.07-3.39] versus 0.39 [95% CI, 0.37-0.41]). A higher percentage of death was seen in the age groups of 55 to 64?years in men (23.11%) and women (21.81%), respectively. In terms of US census regions, the South region has the highest AAMR from cardiovascular deaths with comorbid sarcoidosis compared with other regions (0.78 [95% CI, 0.74-0.82]).
CONCLUSIONS:
The increase of AAMR from cardiovascular deaths with comorbid sarcoidosis and higher cardiovascular mortality rates among adults aged 55 to 64?years highlight the importance of early screening for cardiovascular diseases among patients with sarcoidosis.
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ERS International Congress 2023: highlights from the Interstitial Lung Diseases Assembly.
ERJ Open Res2024 Mar;10(2):. doi: 00839-2023.
Fabbri Laura, Guiot Julien, Vermant Marie, Mi?dlikowska Ewa, Estrella Deborah, Wijsenbeek Marlies S, Wuyts Wim, Bargagli Elena, Froidure Antoine, Spagnolo Paolo, Veltkamp Marcel, Molina-Molina Maria, McCarthy Cormac, Antoniou Katerina, Kreuter Michael, Moor Catharina C,
Abstract
This article summarises a selection of scientific highlights in the field of interstitial lung diseases (ILDs) presented at the International Congress of the European Respiratory Society in 2023. Translational and clinical studies focused on the whole spectrum of ILDs, from (ultra)rare ILDs to sarcoidosis, ILDs associated with connective tissue disease and idiopathic pulmonary fibrosis. The main topics of the 2023 Congress presentations were improving the diagnostic process of ILDs, better prediction of disease course and investigation of novel treatment options.
Copyright ©The authors 2024.
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The usefulness of speckle tracking echocardiography for the prediction of cardiac involvement in patients with biopsy-proven sarcoidosis.
Echocardiography2024 Mar;41(3):e15777. doi: 10.1111/echo.15777.
Jankowska Hanna, Dorniak Karolina, Dudziak Maria, Gli?ska Anna, Sienkiewicz Katarzyna, Kulawiak-Ga??ska Dorota, Fija?kowska Jadwiga, Dubaniewicz Anna, Hellmann Marcin,
Abstract
INTRODUCTION:
Cardiac sarcoidosis (CS) is commonly diagnosed based on clinical criteria and abnormalities in noninvasive imaging reported in patients with biopsy-proven extracardiac sarcoidosis. Electrocardiogram and two-dimensional echocardiography have a low sensitivity for CS detection. Cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) have limitations in terms of cost and availability.
OBJECTIVES:
This study aimed to assess the usefulness of left ventricular longitudinal strain, measured using two-dimensional speckle tracking echocardiography (STE), for the prediction of late gadolinium enhancement (LGE) presence in CMR in patients with biopsy-proven sarcoidosis.
PATIENTS AND METHODS:
A total of 119 patients with biopsy-proven extracardiac sarcoidosis were divided, according to the clinical criteria proposed by the 2014 Heart Rhythm Society expert consensus statement (HRS 2014), into two groups: 43 individuals with "probable cardiac sarcoidosis", CS(+) and 76 individuals without cardiac sarcoidosis, CS (-). Data from echocardiography, CMR, 12-lead ECG and 24 h Holter monitoring were analyzed.
RESULTS:
Left ventricular global longitudinal strain (LV-GLS) was slightly reduced in the entire sarcoidosis group (-18.61± 2.96), no difference between the CS (+) and CS (-) subgroups was found (-18.0% ± 3.2% and -18.9% ± 2.8%, respectively; p = .223). No cut-off value for LV-GLS was identified that could predict the presence of LGE. Segmental longitudinal strain impairment partially correlated with the presence of LGE on CMR.
CONCLUSIONS:
In our cohort of sarcoidosis patients, segmental longitudinal strain proved more helpful in the diagnostic process than LV-GLS. The ultimate role of STE in the diagnosis of CS remains to be established.
© 2024 Wiley Periodicals LLC.
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Kidney manifestations of sarcoidosis.
J Autoimmun2024 Mar;():103207. doi: 10.1016/j.jaut.2024.103207.
Bonella Francesco, Dm Vorselaars Adriane, Wilde Benjamin,
Abstract
Renal involvement is a clinically relevant organ manifestation of sarcoidosis, leading to increased morbidity and complications. Although the exact incidence remains unknown, renal disease is likely to occur in up to one third of all sarcoidosis patients. Every patient with newly diagnosed sarcoidosis should receive a renal work-up and screening for disrupted calcium metabolism. Amid various forms of glomerulonephritis, granulomatous interstitial nephritis is the most common one, but it rarely leads to renal impairment. Histologically, granulomas can be absent. Nephrocalcinosis and nephrolithiasis are frequent forms when hypercalcaemia or hypercalciuria occur. Drugs used for treatment of systemic sarcoidosis can also cause renal damage. Due to its high heterogeneity, renal sarcoidosis can be difficult to treat. Glucocorticoids and various immunosuppressive treatments have been proven to be effective based on case series, but clinical trials are lacking. A treatment guideline for renal sarcoidosis is urgently needed. In this review article, we present an overview of the different forms of renal sarcoidosis and the diagnostic steps to confirm renal involvement; in addition, we provide insights on the management and available treatments. A better understanding regarding the pathogenesis of sarcoidosis is the key for the development of more specific, targeted therapies.
Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.
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Prognostic Value of Combined Assessments of Late Gadolinium Enhancement and Fluorodeoxyglucose Uptake in Cardiac Sarcoidosis.
JACC Cardiovasc Imaging2024 Mar;():. doi: S1936-878X(24)00062-7.
Kazui Sho, Takenaka Sakae, Nagai Toshiyuki, Tsuneta Satonori, Hirata Kenji, Kato Yoshiya, Komoriyama Hirokazu, Kobayashi Yuta, Takahashi Akinori, Kamiya Kiwamu, Temma Taro, Sato Takuma, Tada Atsushi, Yasui Yutaro, Nakai Michikazu, Sato Takahiro, Tsujino Ichizo, Kudo Kohsuke, Konno Satoshi, Anzai Toshihisa,
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Imaging Features of Arrhythmogenic Cardiomyopathies.
Radiographics2024 Apr;44(4):e230154. doi: 10.1148/rg.230154.
Galizia Mauricio S, Attili Anil K, Truesdell William R, Smith Eric D, Helms Adam S, Sulaiman Abdulbaset M A, Madamanchi Chaitanya, Agarwal Prachi P,
Abstract
Arrhythmogenic cardiomyopathy (ACM) is a genetic disease characterized by replacement of ventricular myocardium with fibrofatty tissue, predisposing the patient to ventricular arrhythmias and/or sudden cardiac death. Most cases of ACM are associated with pathogenic variants in genes that encode desmosomal proteins, an important cell-to-cell adhesion complex present in both the heart and skin tissue. Although ACM was first described as a disease predominantly of the right ventricle, it is now acknowledged that it can also primarily involve the left ventricle or both ventricles. The original right-dominant phenotype is traditionally diagnosed using the 2010 task force criteria, a multifactorial algorithm divided into major and minor criteria consisting of structural criteria based on two-dimensional echocardiographic, cardiac MRI, or right ventricular angiographic findings; tissue characterization based on endomyocardial biopsy results; repolarization and depolarization abnormalities based on electrocardiographic findings; arrhythmic features; and family history. Shortfalls in the task force criteria due to the modern understanding of the disease have led to development of the Padua criteria, which include updated criteria for diagnosis of the right-dominant phenotype and new criteria for diagnosis of the left-predominant and biventricular phenotypes. In addition to incorporating cardiac MRI findings of ventricular dilatation, systolic dysfunction, and regional wall motion abnormalities, the new Padua criteria emphasize late gadolinium enhancement at cardiac MRI as a key feature in diagnosis and imaging-based tissue characterization. Conditions to consider in the differential diagnosis of the right-dominant phenotype include various other causes of right ventricular dilatation such as left-to-right shunts and variants of normal right ventricular anatomy that can be misinterpreted as abnormalities. The left-dominant phenotype can mimic myocarditis at imaging and clinical examination. Additional considerations for the differential diagnosis of ACM, particularly for the left-dominant phenotype, include sarcoidosis and dilated cardiomyopathy. RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.
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Cardiac sarcoidosis with extensive and heterogeneous left ventricular FDG uptake in absence of guidelines indication for an implantable defibrillator: Ventricular tachycardia precipitated by immunosuppressive therapy, should we have done differently?
Pacing Clin Electrophysiol2024 Mar;():. doi: 10.1111/pace.14965.
Voisine Emile, Lemay Sylvain, Beaudoin Jonathan, Jacob Philippe, Philippon François, Marchand Laurie, Vallée-Marcotte Bastien, Bernier Florence, Laliberté Claudine, Fortin Sophie, Komlosy Marie-Ève, Birnie David H, Sénéchal Mario,
Abstract
A 40-year-old man, newly diagnosed with cardiac sarcoidosis (CS) presented with symptomatic ventricular tachycardia three days after starting steroid-based immunosuppressive therapy (IT). There was no clear guideline indication for implantable cardioverter-defibrillator (ICD) before the initiation of IT. Shortly after ICD implantation and the initiation of anti-arrhythmic drugs, recurring ventricular arrhythmias required titration of the anti-arrhythmic drug therapy. One-year follow-up assessment showed no significant arrhythmias and complete PET scan FDG uptake suppression. This case, along with recent publications, suggests transient pro-arrhythmic effects of steroids in patients with CS, which are not appropriately addressed in the current guidelines. We believe ICD implantation should be considered in clinically manifest CS before initiating IT, particularly in cases with heterogeneous and/or extensive FDG uptake on PET scans.
© 2024 The Authors. Pacing and Clinical Electrophysiology published by Wiley Periodicals LLC.
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Machine learning approach using F-FDG-PET-radiomic features and the visibility of right ventricle F-FDG uptake for predicting clinical events in patients with cardiac sarcoidosis.
Jpn J Radiol2024 Mar;():. doi: 10.1007/s11604-024-01546-y.
Nakajo Masatoyo, Hirahara Daisuke, Jinguji Megumi, Ojima Satoko, Hirahara Mitsuho, Tani Atsushi, Takumi Koji, Kamimura Kiyohisa, Ohishi Mitsuru, Yoshiura Takashi,
Abstract
OBJECTIVES:
To investigate the usefulness of machine learning (ML) models using pretreatment F-FDG-PET-based radiomic features for predicting adverse clinical events (ACEs) in patients with cardiac sarcoidosis (CS).
MATERIALS AND METHODS:
This retrospective study included 47 patients with CS who underwent F-FDG-PET/CT scan before treatment. The lesions were assigned to the training (n?=?38) and testing (n?=?9) cohorts. In total, 49 F-FDG-PET-based radiomic features and the visibility of right ventricle F-FDG uptake were used to predict ACEs using seven different ML algorithms (namely, decision tree, random forest [RF], neural network, k-nearest neighbors, Naïve Bayes, logistic regression, and support vector machine [SVM]) with tenfold cross-validation and the synthetic minority over-sampling technique. The ML models were constructed using the top four features ranked by the decrease in Gini impurity. The AUCs and accuracies were used to compare predictive performances.
RESULTS:
Patients who developed ACEs presented with a significantly higher surface area and gray level run length matrix run length non-uniformity (GLRLM_RLNU), and lower neighborhood gray-tone difference matrix_coarseness and sphericity than those without ACEs (each, p?0.05). In the training cohort, all seven ML algorithms had a good classification performance with AUC values of?>?0.80 (range: 0.841-0.944). In the testing cohort, the RF algorithm had the highest AUC and accuracy (88.9% [8/9]) with a similar classification performance between training and testing cohorts (AUC: 0.945 vs 0.889). GLRLM_RLNU was the most important feature of the modeling process of this RF algorithm.
CONCLUSION:
ML analyses using F-FDG-PET-based radiomic features may be useful for predicting ACEs in patients with CS.
© 2024. The Author(s).
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New Updates in Sarcoidosis Research: Defining and Renewing the Quest.
Am J Physiol Lung Cell Mol Physiol -
Efficacy and safety of Infliximab in systemic sarcoidosis according to GenPhenReSa organ-involvement phenotype: a retrospective study of 55 patients.
Respir Res2024 Mar;25(1):124. doi: 124.
Rivière Etienne, Jourde Wendy, Gensous Noémie, Demant Xavier, Ribeiro Emmanuel, Duffau Pierre, Mercié Patrick, Viallard Jean-François, Lazaro Estibaliz,
Abstract
BACKGROUND:
Infliximab is currently recommended as a third-line treatment for refractory sarcoidosis. Data in function of clinical phenotype are currently lacking. We evaluated patients' characteristics and responses to infliximab according to their GenPhenReSa cluster.
METHODS:
We evaluated clinical and biological characteristics of patients diagnosed with sarcoidosis who received infliximab between September 2008 and April 2019 at our centre.
RESULTS:
Fifty-five patients (median disease duration, 87 months) received infliximab: 48 (87%) as a second- or third-line treatment, and 7 (13%) as a first-line treatment. After a median duration of 12 months, 24 (45%) and 14 (25%) patients achieved complete and partial responses, respectively, together with a significant decrease in the number of affected organs and tapering of steroid doses. All patients with neurosarcoidosis (OR 17), 90% in group 2 (ocular-cardiac-cutaneous-CNS, OR 7.4), and approximately two-thirds of those in groups 1 (abdominal organs), 4 (pulmonary-lympho-nodal), and 5 (extrapulmonary), achieved a response, whereas patients in group 3 (musculoskeletal-cutaneous) had a treatment-failure OR of 9. Infliximab could be stopped after complete remission was achieved in 7 patients: 4 relapsed after a median of 6 months. Overall, 36% of patients experienced serious adverse events, mainly infections, which led to treatment cessation in 29% of patients and caused two deaths.
CONCLUSIONS:
Other than patients with musculoskeletal-cutaneous involvement (group 3), infliximab led to a good response for patients with CNS (group 2) and liver (group 1) organ-predominant sarcoidosis. However, it led to serious infections and merely suspended sarcoidosis, so further research on factors predictive of relapse is needed.
© 2024. The Author(s).
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A case of cardiac sarcoidosis mimicking acute phase of takotsubo cardiomyopathy evaluated by multimodality cardiac imaging.
J Cardiol Cases2024 Mar;29(3):132-135. doi: 10.1016/j.jccase.2023.11.013.
Takada Yasuyuki, Hida Satoshi, Fujii Masatsune, Yazaki Yoshinao, Satomi Kazuhiro,
Abstract
UNLABELLED:
The patient was a 68-year-old woman who experienced loss of consciousness owing to a seizure while walking and bruised her face. Twelve?lead electrocardiography displayed a complete atrioventricular block. Transthoracic echocardiography displayed hypokinesis from the middle to apex of the myocardium. Emergency coronary angiography displayed no clear stenosis of the coronary arteries, and left ventriculography displayed takotsubo-like abnormal left ventricular wall motion. Tc-sestamibi/I-beta-methyl iodophenyl pentadecanoic acid dual single-photon emission computed tomography displayed a perfusion/metabolism mismatch in the left apex, anterior segment, and inferior segment of the myocardium in the acute phase, which improved in the chronic phase. Similar mismatch findings were observed in the ventricular septum, which persisted in the chronic phase. Blood biomarkers of sarcoidosis were positive. Myocardial delayed enhancement was observed in the mid layer of the basal septum and inferior wall on cardiac magnetic resonance imaging. Fluorodeoxyglucose-positron emission tomography displayed signal accumulation in the basal septum. The clinical course of the patient suggested the possibility of cardiac sarcoidosis combined with takotsubo cardiomyopathy. This is a valuable case in which changes over time were detected by multimodality cardiac imaging.
LEARNING OBJECTIVE:
Cardiac manifestations of both takotsubo cardiomyopathy and sarcoidosis are similar, with both causing abnormal left ventricular wall motion. The co-occurrence of these conditions has been seldomly reported to date. The similarity of the characteristics of each condition poses a challenge in the diagnostic process. The utilization of multimodality cardiac imaging techniques, as demonstrated in the present case, is an effective means of establishing a diagnosis.
© 2023 Japanese College of Cardiology. Published by Elsevier Ltd.
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'The imitation game': a heart failure case report with a great diagnostic twist.
Eur Heart J Case Rep2024 Mar;8(3):ytae107. doi: ytae107.
Khan-Kheil Ayisha Mehtab, Demetriades Polyvios, Steeds Richard P, Moody William E,
Abstract
BACKGROUND:
Arrhythmogenic ventricular cardiomyopathy (AVC) is a hereditary cardiomyopathy that has been associated with mutations in genes encoding for components of the cardiac desmosome including desmoglein-2 (DSG-2).
CASE SUMMARY:
A 49-year-old male presented with decompensated heart failure and ventricular arrythmias. A cardiac magnetic resonance scan demonstrated a dilated left ventricle (LV) with severely impaired systolic function and extensive subepicardial late gadolinium enhancement in the lateral wall. An 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) scan identified myocardial uptake consistent with inflammation. Following treatment with steroids for presumed cardiac sarcoidosis, a repeat FDG-PET confirmed resolution of inflammation. A dilated cardiomyopathy/AVC gene panel, however, subsequently identified a pathogenic variant in the DSG-2 gene.
DISCUSSION:
We describe the case of a patient presenting with clinical and imaging features suggestive for cardiac sarcoidosis, however genetic testing established a diagnosis of DSG-2 associated AVC. DSG-2 mutations in AVC are associated with frequent LV involvement and heart failure. Active inflammation has been observed in other cardiomyopathies, specifically in desmoplakin cardiomyopathy which has a similar clinical course to DSG-2. To our knowledge, this is the first case of DSG-2 cardiomyopathy presenting in this manner. We encourage clinicians to have a high index of suspicion of inflammatory cardiomyopathies as a differential to myocarditis and cardiac sarcoidosis, when patients present with evidence of decompensated heart failure, arrhythmias, and active myocardial inflammation.
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Imaging of Cardiac Sarcoidosis: An Update and Future Aspects.
Semin Nucl Med2024 Mar;():. doi: S0001-2998(24)00016-3.
Saric Petar, Bois John P, Giudicessi John R, Rosenbaum Andrew N, Kusmirek Joanna E, Lin Grace, Chareonthaitawee Panithaya,
Abstract
Cardiac sarcoidosis (CS), an increasingly recognized disease of unknown etiology, is associated with significant morbidity and mortality. Given the limited diagnostic yield of traditional endomyocardial biopsy (EMB), there is increasing reliance on multimodality cardiovascular imaging in the diagnosis and management of CS, with EMB being largely supplanted by the use of F-fluorodeoxyglucose (FDG-PET) and cardiac magnetic resonance imaging (CMR). This article aims to provide a comprehensive review of imaging modalities currently utilized in the screening, diagnosis, and monitoring of CS, while highlighting the latest developments in each area.
Copyright © 2024 Elsevier Inc. All rights reserved.
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Spontaneous Remission Without Steroid Therapy in Isolated Cardiac Sarcoidosis with Severe Left Ventricular Systolic Dysfunction.
Int Heart J2024 Mar;():. doi: 10.1536/ihj.23-399.
Ozawa Takaya, Goto Kenji, Miura Katsuya, Kobayashi Kazunari, Kikuta Yuetsu, Sato Katsumasa, Taniguchi Masahito, Hiramatsu Shigeki, Takebayashi Hideo, Haruta Seiichi,
Abstract
Spontaneous remission is often observed in extracardiac cases of sarcoidosis, such as skin sarcoidosis. However, for cardiac sarcoidosis (CS), the prognosis is unfavorable. Although corticosteroids are the first-line treatment for CS, data regarding the natural history of isolated CS are limited. We describe a rare case of isolated CS with severe left ventricular systolic dysfunction that improved without steroid therapy.
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Endomyocardial Biopsy in Clinical Practice: The Diagnostic Yield and Insights from a 5-Year Single-Center Experience.
Hellenic J Cardiol2024 Mar;():. doi: S1109-9666(24)00059-9.
Karameh Mutaz, Meir Karen, Qadan Abed, Pappo Orit, Cohen Dotan, Durst Ronen, Amir Offer, Asleh Rabea,
Abstract
BACKGROUND AND AIMS:
Endomyocardial biopsy (EMB) is a diagnostic tool for evaluating various cardiac conditions, such as myocarditis and myocardial infiltrative diseases. It is also the gold standard screening technique for detecting allograft rejection after heart transplantation. Despite advances in noninvasive imaging modalities for myocardial tissue characterization, EMB is still necessary for making a definitive diagnosis and determining treatment for certain conditions. Herein, we report our recent experience using EMB and its diagnostic yield.
METHODS AND RESULTS:
We retrospectively reviewed EMBs performed at our institution from March 2018 through March 2023. Clinical data, including patient characteristics, indication and diagnostic yield of EMB, and procedure-related complications were collected. Histopathological findings of the biopsies were recorded and classified based on the degree to which they matched the clinical diagnosis and cardiac magnetic resonance imaging (CMR) findings. A total of 212 EMBs obtained in 178 consecutive patients were retrospectively analyzed, with 42 biopsies performed for allograft rejection surveillance (10 patients) and the remaining performed for presumptive diagnosis of acute myocarditis or unexplained cardiomyopathy. Among the non-heart transplant cases, 54.7% of EMBs provided a clear diagnosis. The most common diagnosis was myocarditis (69%), followed by cardiac amyloidosis (CA) (26%). EMB was also helpful in detecting several rare cardiac conditions, such as eosinophilic granulomatosis with polyangiitis (EGPA), Fabry disease, and cardiac sarcoidosis. In a cohort of 101 patients who underwent both CMR and EMB, the results were concordant in 66% of cases. However, in 24.7% of patients, EMB was able to identify pathological conditions where CMR results were inconclusive, highlighting its complementary role in determining the accurate diagnosis. No complications were reported in any of the 212 EMBs performed.
CONCLUSIONS:
With advances in cardiac imaging modalities, EMB is not routinely indicated for the diagnosis of cardiomyopathy. However, EMB is still an important tool for diagnosing specific cardiac diseases and could be crucial for confirming the diagnosis. EMB is generally safe if performed at experienced centers.
Copyright © 2024 Hellenic Society of Cardiology. Published by Elsevier Inc. All rights reserved.
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Role of serial FDG-PET in determining the therapeutic efficacy of immunosuppression and clinical outcome in patients with cardiac sarcoidosis.
J Nucl Cardiol2024 Mar;():101842. doi: 10.1016/j.nuclcard.2024.101842.
Okafor Joseph, Khattar Rajdeep, Kouranos Vasileios, Ohri Shreya, Diana Davide, Ebeke Ehis, Azzu Alessia, Ahmed Raheel, Wells Athol, Baksi A John, Sharma Rakesh, Wechalekar Kshama,
Abstract
BACKGROUND:
Myocardial inflammation and perfusion defects detected by 18F-fludeoxyglucose (FDG) and Rubidium-82 positron emission tomography (PET) may be associated with ventricular arrhythmias (VA) in cardiac sarcoidosis (CS). The role of serial quantitative PET in determining the effect of treatment on myocardial inflammation and clinical outcomes is yet to be defined.
METHODS:
Newly diagnosed CS patients with active myocardial inflammation (SUVmax ?2.5) were treated with immunosuppression, then underwent repeat FDG-PET, Rubidium-82 and echocardiographic imaging 6-12 months later. Serial changes in SUVmax, SUVmean, inflammatory extent, perfusion defect (PD) extent, metabolism/perfusion mismatch extent, global cardiac metabolic activity and left ventricular ejection fraction (LVEF) were assessed. The primary endpoint was a composite of all-cause mortality, serious VA and HF hospitalization. Event data was recorded from the date of the second FDG-PET.
RESULTS:
The study population consisted of 113 patients (66% male, age 55±11 years, LVEF 54±13%). SUVmax reduced from 4.5 (IQR 3.3-7.1) to 2.7 (2.2-3.6). Overall, 94 (83%) patients saw serial reduction in SUVmax with 42 (37%) demonstrating complete response (SUVmax 10%.
CONCLUSION:
In our cohort with active CS, following a treatment-induced reduction in myocardial inflammation, PD extent was the main predictor of adverse events.
Copyright © 2024. Published by Elsevier Inc.
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