Pubblicazioni recenti - cardiac sarcoidosis
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No beneficial use of the wearable cardioverter defibrillator among patients suffering from inherited and congenital heart disease: data from a European multicenter registry.
Front Cardiovasc Med2024 ;11():1384736. doi: 1384736.
Koepsel Katharina, Dreher Tobias C, Blockhaus Christian, Gotzmann Michael, Klein Norbert, Kuntz Thomas, Shin Dong-In, Lapp Hendrik, Schiedat Fabian, Abumayyaleh Mohammad, Beiert Thomas, Weth Christian, Kovacs Boldizsar, Rosenkaimer Stephanie, Kowitz Jacqueline, Saguner Ardan Muammer, Erath Julia W, Duru Firat, Mügge Andreas, Akin Ibrahim, Aweimer Assem, Hamdani Nazha, El-Battrawy Ibrahim,
Abstract
BACKGROUND:
Data on the use of the wearable cardioverter defibrillator in patients suffering from inherited and congenital heart disease are limited. Consequently, evidence for guideline recommendations in this patient population is lacking.
METHODS:
In total 1,675 patients were included in a multicenter registry of eight European centers. In the present cohort, we included 18 patients suffering from congenital and inherited heart disease.
RESULTS:
Nine patients (50%) were male with a mean age of 41.3?±?16.4 years. Four patients suffered from hypertrophic cardiomyopathy (HCM), four patients suffered from non-compaction cardiomyopathy (NCCM), two patients were diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) and one patient suffered from muscular dystrophy of the limb-girdle type with cardiac involvement, secondary cardiomyopathy. Three patients presented with Brugada syndrome (BrS). One patient suffered from long-QT syndrome type 1 (LQTS1). Furthermore, two patients had congenital heart defects and one patient suffered from cardiac sarcoidosis (CS). There were no appropriate/inappropriate shocks with the WCD in this cohort. One patient had recurrent self-limiting sustained ventricular tachycardia during the wear time, but actively inhibited a shock and was hospitalized. The compliance rate in this cohort was 77.8% with a mean wear time of 45.3?±?26.9 days with a mean follow-up time of 570?±?734 days. 55.6% (10/18) of the patients received an ICD after WCD wear time.
CONCLUSIONS:
This retrospective study of patients with inherited and congenital heart disease shows that WCD use is not beneficial in the majority of patients with inherited and congenital heart disease.
© 2024 Koepsel, Dreher, Blockhaus, Gotzmann, Klein, Kuntz, Shin, Lapp, Schiedat, Abumayyaleh, Beiert, Weth, Kovacs, Rosenkaimer, Kowitz, Saguner, Erath, Duru, Mügge, Akin, Aweimer, Hamdani and El-Battrawy.
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Effect of exercise on cluneal nerve entrapment neuropathy: a case report.
J Med Case Rep2024 Jul;18(1):338. doi: 10.1186/s13256-024-04641-w.
Özüberk Burcu, Deniz Mine Argal?, Soyupek Feray Cinevre,
Abstract
BACKGROUND:
Low back pain is an important disability problem frequently encountered in the clinic. In the literature, it has been shown that neuropathic pain in chronic low back pain is quite common in patients. Although superior cluneal nerve entrapment syndrome is an underdiagnosed cause of low back and leg pain, differential diagnosis is very important anatomically and clinically. The superior cluneal nerves are pure sensory nerves that innervate the skin of the upper part of the buttocks. In the literature, methods such as surgery, nerve blockade, prolotherapy, and acupuncture have been used in the treatment of cluneal nerve entrapment syndrome, but there are no studies on exercise. In this case report, our aim is to explain the importance of differential diagnosis in cluneal nerve entrapment syndrome, which is one of the common causes of low back pain in the clinic, and the effects of exercise in this disease.
CASE PRESENTATION:
A 22-year-old, Turkish-ethnicity, male patient with complaints of low back pain, neck-back pain, and weakness did not use alcohol or cigarettes. In his family history, there was a history of diabetes in the mother and diabetes and heart failure in the father. He had a history of osteoporosis, epilepsy, asthma, sarcoidosis, and cardiac arrhythmia. The patient reported that he suffered from constipation three to four times a month. As a result of the detailed evaluation, the planned exercise prescription was taught to the patient, and after it was confirmed that the patient did the exercises correctly for 3 days, the exercise brochure was given and followed as a home exercise program for 8 weeks.
CONCLUSIONS:
Lumbar stabilization exercises, gluteal muscle strengthening exercises, thoracolumbar fascia mobilization, and stretching exercises, which will be given in accordance with the clinical anatomy of the disease after the correct diagnosis in cluneal nerve entrapment syndrome, have been beneficial for the patient. However, we think that randomized controlled studies with a large sample will contribute to the literature.
© 2024. The Author(s).
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Standardized ketogenic dietary preparation for metabolic PET imaging in suspected and known cardiac sarcoidosis.
Eur Heart J Imaging Methods Pract2024 Jan;2(1):qyae037. doi: qyae037.
Hutt Erika, Goldar Ghazaleh, Jaber Wael A, Cremer Paul C,
Abstract
AIMS:
A major limitation of cardiac positron emission tomography (PET) with F18-fluorodeoxyglucose (F18-FDG) for the evaluation of cardiac sarcoidosis (CS) is associated with physiologic myocardial glucose uptake. The optimal dietary protocol to suppress physiologic myocardial F18-FDG uptake is not well-established. We aimed to evaluate the diagnostic performance of a novel dietary preparation using a ketone-based infant formula.
METHODS AND RESULTS:
Between 2018 and 2021, consecutive studies using a ketogenic dietary preparation were identified ( = 198). The rate of non-diagnostic studies due to failure to suppress myocardial glucose was 7.1% ( = 14) with a similar incidence in diabetics ( = 6, 8.1%). Among studies reported to have no inflammation ( = 137), 130 studies (66%) had mean myocardial standardized uptake value (SUV) less than or equal to mean blood pool SUV.
CONCLUSION:
Patient preparation with a ketone-based infant formula resulted in low rate of inappropriate myocardial glucose suppression in patients undergoing F18-FDG cardiac PET to evaluate CS.
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Subepicardial hypoattenuation revealed by computed tomography angiography in a patient with cardiac sarcoidosis.
Eur Heart J Imaging Methods Pract2024 Jan;2(1):qyae023. doi: qyae023.
Piciucchi S, Gardini E, Dallaserra C, De Vita M, Poletti V, Ravaglia C,
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Somatostatin receptors in fibrotic myocardium.
PLoS One2024 ;19(7):e0304813. doi: e0304813.
Castillero Estibaliz, Camillo Chiara, Erwin W Clinton, Singh Sameer, Mohamoud Nafisa, George Isaac, Eapen Elizabeth, Dockery Keith, Ferrari Giovanni, Gupta Himanshu,
Abstract
A patient with a neuroendocrine tumor and history of coronary artery disease underwent PET with 68Ga-DOTATATE PET tracer for tumor visualization. Analysis of the scan showed uptake of 68Ga-DOTATATE in the left ventricle corresponding to previous myocardial infarct. 68Ga-DOTATATE binds by somatostatin receptors (SSTR) and it has been proposed that it may be useful for the detection of cardiac inflammatory lesions. We aimed to test whether SSTR could be upregulated in cardiac fibrotic scar. We analyzed SSTR in cardiac samples from patients with end-stage ischemic cardiomyopathy (ICM, n = 8) and control hearts (n = 5). In mature ICM tissue, SSTR1 and SSTR2 expression was unchanged and SSTR5 expression was significantly decreased in ICM samples vs. control. Immunohistochemistry showed increased SSTR1 and SSTR2 in ICM. Areas with SSTR1 or SSTR2 staining were often adjacent to fibrotic areas. The majority of SSTR1 and SSTR2 staining localized in cardiomyocytes in fibrotic scar-rich areas where CD68 macrophage staining was not present. SSTR are occasionally upregulated in cardiac fibrotic areas. When using 68Ga-DOTATATE PET tracer to detect cardiac sarcoidosis or atherosclerotic plaque, the possibility of tracer uptake in fibrotic areas should be considered.
Copyright: © 2024 Castillero et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Isolated Cardiac Sarcoidosis Presenting as High-Degree Atrioventricular Block.
Cureus2024 Jun;16(6):e62685. doi: e62685.
Owusu Ryian, Alakhras Hazem, Strubchevska Kateryna, Walsh Daniel G,
Abstract
Isolated cardiac sarcoidosis is a rare phenomenon of systemic sarcoidosis, with presentation ranging from asymptomatic to sudden cardiac death. Controversy exists on diagnostic and therapeutic options, creating an ongoing challenge for clinicians in providing patient care. A 79-year-old male presented status post looposcopy and interval ureteral stent replacement with sinus bradycardia and high-degree atrioventricular block. A comprehensive examination was performed that conclusively ruled out common etiologies of atrioventricular block, including coronary artery disease, electrolyte abnormalities, and medications. This prompted an investigation using advanced cardiac imaging modalities that demonstrated cardiac sarcoidosis. Computed tomography of the chest was negative for lymphadenopathy or infiltrates indicative of pulmonary involvement. The lack of extracardiac manifestations, in combination with imaging findings, led to a probable diagnosis of isolated cardiac sarcoidosis. The patient underwent biventricular implantable cardioverter defibrillator placement and was started on oral corticosteroids.
Copyright © 2024, Owusu et al.
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Current uses and understanding of PET imaging in cardiac sarcoidosis.
Am J Nucl Med Mol Imaging2024 ;14(3):161-174. doi: 10.62347/NANX3492.
Madiraju Alekhya, Bhattaru Abhijit, Pham Truongan, Pundyavana Anish, Rojulpote Krishna Vamsi, Raynor William Y, Werner Thomas J, Alavi Abass,
Abstract
Sarcoidosis is a systemic disease with unclear etiology characterized by the accumulation of noncaseating, immune granulomas in affected tissues. In cardiac sarcoidosis (CS), white blood cells build up within the heart muscles, causing cardiac abnormalities. Accurate and early diagnosis of CS proves challenging. However, usage of positron emission tomography (PET) imaging, namely F-FDG-PET, has proven successful in diagnosing inflammatory cardiomyopathy. This review seeks to examine the role of PET in managing ventricular tachycardia in cardiac sarcoidosis. PET, in conjunction with cardiac magnetic resonance imaging (CMR) is also endorsed as the premier method for diagnosis and management of arrhythmias associated with CS by The Heart Rhythm Society. After a CS diagnosis, risk stratification of ventricular arrhythmias is a necessity given the potential for sudden cardiac death. F-FDG-PET has been successful in monitoring disease advancement and treatment responses in CS patients. Early stages of CS are often treated with immunosuppression drugs if there are additional signs of VT. Currently, corticosteroid and anti-arrhythmia compounds: methotrexate, cyclophosphamide, infliximab, amiodarone, and azathioprine are used to suppress inflammation. F-FDG-PET has certainly proven to be an incredibly useful and accurate diagnostic tool of CS. While late gadolinium enhancement by CMR is efficient in detecting myocardial necrosis and/or advanced fibrosis scarring, F-FDG portrays the increased uptake level of glucose metabolism. In combination PET/MRI has proven to be more successful in improving the efficacy of both scans, addressing their drawbacks, and highlighting their advantages. Managing CS patients is highly involved in detecting inflammatory regions of the heart. Early recognition prevents cardiac abnormality, mainly VT and VF in CS patients, and extends lifespan.
AJNMMI Copyright © 2024.
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Impact of extracardiac sarcoidosis on clinical outcomes in patients with cardiac sarcoidosis: Importance of continued screening for cardiac involvement.
Int J Cardiol2024 Jul;():132368. doi: 10.1016/j.ijcard.2024.132368.
Takaya Yoichi, Nakagawa Koji, Miyoshi Toru, Nishii Nobuhiro, Morita Hiroshi, Nakamura Kazufumi, Yuasa Shinsuke,
Abstract
BACKGROUND:
The prognostic impact of extracardiac sarcoidosis remains unknown in cardiac sarcoidosis (CS). We aimed to evaluate the influence of extracardiac sarcoidosis on clinical outcomes and the effect of continued outpatient visits for screening of cardiac involvement.
METHODS:
Ninety-nine patients with CS were divided into two groups: patients with systemic CS who had prior extracardiac sarcoidosis, patients with isolated CS who had no prior extracardiac sarcoidosis. Patients with systemic CS were divided according to the continuation of outpatient visits. The endpoint was cardiac death, fatal ventricular arrhythmia, or hospitalization for heart failure.
RESULTS:
At the time of diagnosing CS, patients with isolated CS had a higher prevalence of high-grade atrioventricular block or fatal ventricular arrhythmia, and left ventricular contractile dysfunction than those with systemic CS. Over a median follow-up of 42?months, cardiac events occurred in 19 (37%) of 52 patients with systemic CS and in 27 (57%) of 47 patients with isolated CS. The event-free survival rate was worse in patients with isolated CS than in those with systemic CS. Cox proportional hazard analysis showed that the absence of prior extracardiac sarcoidosis was an independent predictor of adverse outcomes. Patients with systemic CS who ceased outpatient visits had a lower left ventricular ejection fraction with severe heart failure symptoms and a worse event-free survival rate than those who continued outpatient visits.
CONCLUSIONS:
The presence of extracardiac sarcoidosis is associated with clinical outcomes. The cessation of screening for cardiac involvement after diagnosing extracardiac sarcoidosis is associated with adverse outcomes.
Copyright © 2024. Published by Elsevier B.V.
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Cluster analysis of blood biomarkers to identify molecular patterns in pulmonary fibrosis: assessment of a multicentre, prospective, observational cohort with independent validation.
Lancet Respir Med2024 Jul;():. doi: S2213-2600(24)00147-4.
Fainberg Hernan P, Moodley Yuben, Triguero Isaac, Corte Tamera J, Sand Jannie M B, Leeming Diana J, Karsdal Morten A, Wells Athol U, Renzoni Elisabetta, Mackintosh John, Tan Dino B A, Li Roger, Porte Joanne, Braybrooke Rebecca, Saini Gauri, Johnson Simon R, Wain Louise V, Molyneaux Philip L, Maher Toby M, Stewart Iain D, Jenkins R Gisli,
Abstract
BACKGROUND:
Pulmonary fibrosis results from alveolar injury, leading to extracellular matrix remodelling and impaired lung function. This study aimed to classify patients with pulmonary fibrosis according to blood biomarkers to differentiate distinct disease patterns, known as endotypes.
METHODS:
In this cluster analysis, we first classified patients from the PROFILE study, a multicentre, prospective, observational cohort of individuals with incident idiopathic pulmonary fibrosis or non-specific interstitial pneumonia in the UK (Nottingham University Hospitals, Nottingham; and Royal Brompton Hospital, London). 13 blood biomarkers representing extracellular matrix remodelling, epithelial stress, and thrombosis were measured by ELISA in the PROFILE study. We classified patients by unsupervised consensus clustering. To evaluate generalisability, a machine learning classifier trained on biomarker signatures derived from consensus clustering was applied to a replication dataset from the Australian Idiopathic Pulmonary Fibrosis Registry (AIPFR). Biomarker associations with mortality and change in percentage of predicted forced vital capacity (FVC%) were assessed, adjusting for age, gender, baseline FVC%, and antifibrotic treatment and steroid treatment before and after baseline. Mortality risk associated with the clusters in the PROFILE cohort was evaluated with Cox proportional hazards models, and mixed-effects models were used to analyse how clustering was associated with longitudinal FVC% in the PROFILE and AIPFR cohorts.
FINDINGS:
455 of 580 participants from the PROFILE study (348 [76%] men and 107 [24%] women; mean age 72·4 years [SD 8·3]) were included in the analysis. Within this group, three clusters were identified based on blood biomarkers. A basement membrane collagen (BM) cluster (n=248 [55%]) showed high concentrations of PRO-C4, PRO-C28, C3M, and C6M, whereas an epithelial injury (EI) cluster (n=109 [24%]) showed high concentrations of MMP-7, SP-D, CYFRA211, CA19-9, and CA-125. The third cluster (crosslinked fibrin [XF] cluster; n=98 [22%]) had high concentrations of X-FIB. In the replication dataset (117 of 833 patients from AIPFR; 87 [74%] men and 30 [26%] women; mean age 72·9 years [SD 7·9]), we identified the same three clusters (BM cluster, n=93 [79%]; EI cluster, n=8 [7%]; XF cluster, n=16 [14%]). These clusters showed similarities with clusters in the PROFILE dataset regarding blood biomarkers and phenotypic signatures. In the PROFILE dataset, the EI and XF clusters were associated with increased mortality risk compared with the BM cluster (EI vs BM: adjusted hazard ratio [HR] 1·88 [95% CI 1·42-2·49], p
INTERPRETATION:
Blood biomarker clustering in pulmonary fibrosis identified three distinct blood biomarker signatures associated with lung function and prognosis, suggesting unique pulmonary fibrosis biomarker patterns. These findings support the presence of pulmonary fibrosis endotypes with the potential to guide targeted therapy development.
FUNDING:
None.
Copyright © 2024 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 license. Published by Elsevier Ltd.. All rights reserved.
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Isolated cardiac sarcoidosis presenting as transient ischemic attack.
Clin Case Rep2024 Jul;12(7):e9035. doi: e9035.
Vasquez Moises A, Andrade-Bucknor Sharon,
Abstract
KEY CLINICAL MESSAGE:
Isolated cardiac sarcoidosis may rarely present with TIA or stroke as an initial clinical manifestation. This case highlights the necessity of a broad differential and a high degree of suspicion for cardiac sarcoidosis in a patient with new neurologic symptoms and evidence of cardiac disease.
ABSTRACT:
Cardiac sarcoidosis is a rare disease with a variety of clinical manifestations including heart failure and sudden death. Stroke as the earliest sign of disease has been described in rare cases. We present a case of a 54-year-old female with recurrent transient ischemic attacks (TIAs) of unknown etiology, initially in the absence of left ventricular dysfunction. Cardiomyopathy was later identified on echocardiography after a second TIA. Cardiac MRI was remarkable for focal left ventricular wall thinning with akinesis and dyskinesis of multiple wall segments, a right ventricular aneurysm, and diffuse myocardial late gadolinium enhancement. PET/CT showed multifocal areas of myocardial FDG uptake. At follow-up, echocardiography showed a left ventricular apical thrombus, in a previously identified thinned, akinetic region, suggesting cardioembolic origin for previous TIAs. She was started on anticoagulation therapy, prednisone, methotrexate, and adalimumab, with resolution of the thrombus and improvement in cardiac function. In conclusion, this case highlights the need to consider CS as a potential cause of cerebrovascular ischemic events in patients with few stroke risk factors but findings indicative of cardiac disease. It is essential to further explore the mechanisms behind these events and develop treatments that target their causes in this patient population.
© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.
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Racial variability in immune responses only partially explains differential systemic sclerosis disease severity.
Ann Rheum Dis2024 Jul;():. doi: ard-2023-225458.
Kuchinad Kamini E, Kim Ji Soo, Woods Adrianne, Leatherman Gwen, Gutierrez-Alamillo Laura, Mayes Maureen D, Domsic Robyn, Ramos Paula S, Silver Richard M, Varga John, Saketkoo Lesley Ann, Kafaja Suzanne, Shanmugan Victoria K, Gordon Jessica, Chung Lorinda, Bernstein Elana J, Gourh Pravitt, Boin Francesco, Kastner Daniel L, Zeger Scott L, Casciola-Rosen Livia, Wigley Fredrick M, Shah Ami A,
Abstract
OBJECTIVE:
To understand if autoantibodies account for racial variation in disease severity, we compared autoantibody distribution and associated phenotype between self-identified black and white systemic sclerosis (SSc) patients.
METHODS:
803 black and 2178 white SSc patients had systematic testing for autoantibodies using Euroimmun (centromere (ACA), RNA-polymerase III (POLR3), Scl70, PM/Scl, NOR90, Th/To, Ku, U3RNP and Ro52) and commercial ELISA (U1RNP). In this observational study, logistic regression was performed to assess the association between self-identified race and outcomes, adjusting for autoantibodies. To estimate whether the effect of race was mediated by autoantibody status, race coefficients from multivariate models including and excluding autoantibodies were compared.
RESULTS:
Anti-Scl70, anti-U1RNP, anti-U3RNP, anti-Th/To, anti-Ku and anti-NOR90 were more common in the black cohort than in the white cohort, which was enriched for ACA, anti-POLR3 and anti-PM/Scl. Black individuals had a higher prevalence of severe Raynaud's, skin, lung, gastrointestinal and renal disease whereas white individuals had a higher prevalence of severe heart and muscle disease. Adjusting for autoantibodies decreased the effect of race on outcome for telangiectasias, forced vital capacity
CONCLUSIONS:
This study is the largest systematic analysis of autoantibody responses in a geographically diverse population of black SSc patients. Black and white individuals with SSc have distinct autoantibody profiles. Autoantibodies explain only a fraction of the effect of race on clinical outcomes, suggesting other factors contribute to disparate outcomes between these groups.
© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ on behalf of EULAR.
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Clinical Outcomes of Transcatheter Aortic Valve Replacement (TAVR) vs Surgical Aortic Valve Replacement (SAVR) in Patients With Sarcoidosis.
Cureus2024 Jun;16(6):e62477. doi: e62477.
Khan Muhammad Z, Alvarez Rene, Bhuiyan Mohammad Alfrad Nobel, Faisal Abu Saleh Mosa, O'Neill Parker, Siddiqui Muhammad, Kaki Praneet, Franklin Sona, Waqas Muhammad, Shah Hadia, Kanawati Eyad I, Murtaza Mohammed,
Abstract
Introduction Data regarding clinical outcomes after transcatheter aortic valve replacement (TAVR) vs surgical aortic valve replacement (SAVR) in patients with sarcoidosis is lacking. This study aims to clarify the clinical outcomes of TAVR vs SAVR in patients with sarcoidosis. Methods Data was collected from the National Inpatient Sample database from 2016-2019 using validated ICD-10-CM codes for sarcoidosis, TAVR, and SAVR. Patients were divided into two cohorts: those who underwent TAVR and those who underwent SAVR. Statistical analysis was performed using Pearson's chi-squared test to determine clinical outcomes of TAVR vs SAVR in patients with sarcoidosis. Results The prevalence of sarcoidosis was 0.23% among total study patients (n=142,420,378). After exclusions, the prevalence of TAVR was 650 (49%) and SAVR was 675 (51%) in patients with sarcoidosis. Patients who underwent TAVR were on average older (74 vs 65 years old, p=0.001), and more likely to be female (57 vs 40%, p
Copyright © 2024, Khan et al.
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Cardiac Sarcoidosis: Utilizing Cardiac MRI and PET-CT.
Curr Cardiol Rep2024 Jul;():. doi: 10.1007/s11886-024-02093-8.
Ositelu Kamari, Abraham Sonu, Okwuosa Ike S,
Abstract
PURPOSEOF REVIEW:
Cardiac sarcoidosis is an inflammatory condition that has been associated with deleterious cardiac manifestations. The diagnosis of cardiac sarcoidosis is challenging and can be guided by advanced cardiac imaging.
RECENT FINDINGS:
Endomyocardial biopsy lacks sensitivity in confirming a diagnosis of cardiac sarcoidosis. Studies have shown that the use of cardiac magnetic resonance imaging (MRI) and cardiac Positron Emission Testing (PET) are associated with increased sensitivity and specificity in the diagnosis of cardiac sarcoidosis. Cardiac MRI and cardiac PET CT, although distinct entities, are complimentary in the diagnosis, prognostication of major cardiac events, and aid in the treatment algorithm in patients with cardiac sarcoidosis.
© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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Prognostic role of late gadolinium-enhanced MRI in confirmed and suspected cardiac sarcoidosis: meta-analysis.
Int J Cardiovasc Imaging2024 Jul;():. doi: 10.1007/s10554-024-03191-y.
Sekii Ryusuke, Kato Shingo, Horita Nobuyuki, Utsunomiya Daisuke,
Abstract
The prognostic implications of late gadolinium-enhanced (LGE) magnetic resonance imaging (MRI) in the context of cardiac sarcoidosis (CS) have attracted considerable attention. Nevertheless, a subset of studies has undistinguished confirmed and suspected CS cases, thereby engendering interpretative ambiguities. In this meta-analysis, we evaluated the differences in cardiac MRI findings and their prognostic utility between confirmed and suspected CS. A literature search was conducted using PubMed, Web of Science, and Cochrane libraries to compare the findings of cardiac MRI and its prognostic value in CS and suspected CS. A meta-analysis was performed to compare the prevalence of LGE MRI, odds ratios, and hazard ratios for predicting cardiac events in both groups. A total of 21 studies encompassing 24 different populations were included in the meta-analysis (CS: 393 cases, suspected CS: 2151 cases). CS had a higher frequency of LGE of the left ventricle (87.2% vs. 36.4%, p?0.0001) and right ventricle (62.1% vs. 23.8%, p?=?0.04) than suspected CS. In patients with suspected CS, the presence of left ventricular LGE was associated with higher all-cause mortality [odds ratio: 5.70 (95%CI: 2.51-12.93), p?0.0001, I?=?8%, p for heterogeneity?=?0.37] and ventricular arrhythmia [odds ratio: 15.51 (95%CI: 5.65-42.55), p?0.0001, I?=?0, p for heterogeneity?=?0.94]. In contrast, in CS, not the presence but extent of left ventricular LGE was a significant predictor of outcome (hazard ratio?=?1.83 per 10% increase of %LGE (95%CI: 1.43-2.34, p?0.001, I?=?15, p for heterogeneity?=?0.31). The presence of left ventricular LGE was a strong prognostic factor in suspected sarcoidosis. However, the extremely high prevalence of left ventricular LGE in confirmed CS suggests that the quantitative assessment of LGE is useful for prognostic estimation.
© 2024. The Author(s), under exclusive licence to Springer Nature B.V.
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A rare case of pericardial sarcoidosis presenting as chest pain.
Clin Case Rep2024 Jul;12(7):e9160. doi: e9160.
El Sharu Husam, Jain Prarthana, Singer Bart, Snyder E Amanda,
Abstract
KEY CLINICAL MESSAGE:
Pericardial sarcoidosis is an uncommon cause of chest pain to consider, and it requires a heightened level of suspicion and thorough history gathering. If there is suspicion of inflammatory disease, pursuing advanced imaging and biopsies is crucial, as early immunosuppressive treatment can enhance outcomes.
ABSTRACT:
Pericardial involvement in sarcoidosis is a rare condition with limited research. This case study discusses a 52-year-old African American woman who presented with subacute chest pain and was diagnosed with pericardial sarcoidosis. Diagnostic evaluation revealed extensive lymphadenopathy and pericardial effusion, and a pericardial biopsy confirmed non-caseating granulomatous inflammation. Treatment with steroids and methotrexate resulted in clinical improvement. Eight?months follow-up showed near resolution of pericardial disease. This case emphasizes the importance of considering cardiac sarcoidosis in sarcoidosis patients, utilizing advanced imaging for accurate diagnosis, and tailoring treatment to the level of cardiac involvement.
© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.
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Clinical Outcomes with Cardiac Resynchronization Therapy in Patients with Cardiac Sarcoidosis: A Systematic Review and Proportional Meta-analysis.
Curr Probl Cardiol2024 Jul;():102747. doi: 10.1016/j.cpcardiol.2024.102747.
Ahmed Raheel, Sivasankaran Karthikeyan, Ahsan Areeba, Mactaggart Sebastian, Azzu Alessia, Dulay Mansimran Singh, Ramphul Kamleshun, Liu Alexander, Okafor Joseph, Dragon Margaux, Kouranos Vasilis, Ahmed Mushood, Sharma Rakesh,
Abstract
BACKGROUND:
Cardiac sarcoidosis (CS) is an inflammatory condition that can present with heart failure (HF). Cardiac resynchronization therapy (CRT) is known to improve clinical outcomes for patients with left bundle branch block in the general HF population. However, data about the outcomes of CRT in CS is limited.
METHODS:
A systematic literature search was conducted using PubMed/Medline, Embase, and the Cochrane Library from inception to February 2024 to identify studies that reported clinical outcomes following the use of CRT in patients with CS. Data for outcomes was extracted, pooled, and analyzed. OpenMetaAnalyst was used for pooling untransformed proportions along with the corresponding 95% confidence intervals (CIs).
RESULTS:
Five studies with a total of 176 CS patients who received CRT were included. The pooled incidence for all-cause mortality was 7.6% (95% CI: -3% to 18%), for HF-related hospitalizations 23.2% (95% CI: 2% to 43%), and for major adverse cerebral and cardiovascular events 27% (95% CI: 8% to 45%) after a mean follow-up of 60.1 (±48.7) months. The pooled left ventricular ejection fraction (LVEF) was 34.28% (95% CI: 29.88% to 38.68%) demonstrating an improvement of 3.75% in LVEF from baseline LVEF of 30.58% (95% CI: 24.68% to 36.48%). The mean New York Heart Association (NYHA) functional class was 2.16 (95% CI: 1.47 to 2.84) after CRT as compared to the baseline mean NYHA of 2.58 (95% CI: 2.29 to 2.86).
CONCLUSION:
Although improvements were observed in LVEF and mean NYHA, mortality was high in CS patients with CRT.
Copyright © 2024. Published by Elsevier Inc.
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Reevaluating Diagnosis of Sarcoidosis: Biopsy with Necrosis in Mycobacterial Endemic Areas.
J Assoc Physicians India2024 Jul;72(7):94-96. doi: 10.59556/japi.72.0509.
Manwatkar Abhilasha A, Das John Kumar, Issac N P Rijo, Kothapalli Nagamounika, Chandhu A S, Prabhu V, Mathew John,
Abstract
BACKGROUND:
Sarcoidosis is a multisystem inflammatory disease with a variable presentation. The most characteristic feature of sarcoidosis is nonnecrotizing granulomas. However, when sarcoidosis presents with rare organ involvement, and biopsy shows necrosis, the diagnosis becomes challenging.
CASE PRESENTATION:
Here, we present three cases of sarcoidosis with unusual organ involvement and biopsy findings of necrosis, leading to a delay in diagnosis and treatment. Case 1 was presented with lymphoreticular involvement within the intraparotid lymph node and genitourinary area. Biopsy from the epididymis showed necrosis, initially leading to treatment for tuberculosis (TB). Case 2 describes lymphoreticular involvement and cardiac symptoms. His cervical and bone marrow biopsies showed necrosis. Case 3's presentation was disseminated lymphadenopathy with hepatosplenomegaly, initially suspected as malignancy or TB.
CONCLUSION:
While biopsy plays a significant role in diagnosing sarcoidosis, the presence of necrosis alone should not lead to its exclusion.
© Journal of the Association of Physicians of India 2024.
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Can FDG-PET Imaging Identify Cardiac Sarcoidosis Disease Phenotypes?
Curr Cardiol Rep2024 Jul;():. doi: 10.1007/s11886-024-02086-7.
Boczar Kevin Emery, Park Yooyhun, Wiefels Christiane,
Abstract
PURPOSE OF REVIEW:
Despite the scarcity of data, most guidelines have advocated for the treatment of cardiac sarcoidosis with corticosteroids. However, there is heterogeneity in disease presentation and response to treatment, which can make treatment challenging. The ability to identify disease phenotypes to allow for tailored therapy is therefore highly desirable. This review will seek to outline the disease phenotypes of cardiac sarcoidosis and the role that FDG-PET imaging can play in identifying these phenotypes to optimize disease diagnosis and treatment management.
RECENT FINDINGS:
FDG PET can identify cardiac sarcoidosis and is being increasingly used to monitor therapeutic response to immunosuppressive therapy, to follow treatment response after discontinuation of corticosteroid therapy, and to evaluate for disease relapse. Modern quantitative techniques using FDG PET imaging may allow for even better phenotypic disease characterization and the ability to track the response to immunosuppression more accurately. FDG PET currently plays an important role in cardiac sarcoidosis diagnosis. However, it also affords us the opportunity to offer insights into cardiac sarcoidosis disease phenotypes to better understand the underlying disease process and in the future may allows us to tailor therapies accordingly.
© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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Diagnostic Value of Late Gadolinium Enhancement at Cardiac Magnetic Resonance to distinguish Arrhythmogenic Right Ventricular Cardiomyopathy from Differentials.
J Cardiovasc Magn Reson2024 Jul;():101059. doi: 10.1016/j.jocmr.2024.101059.
Rekker Lian Y, Muller Steven A, Gasperetti Alessio, Bourfiss Mimount, Oerlemans Marish Ifj, Cramer Maarten J, Zimmerman Stefan L, Dooijes Dennis, Schalkx Hanke, van der Harst Pim, James Cynthia A, Peter van Tintelen J, Guglielmo Marco, Velthuis Birgitta K, Te Riele Anneline Sjm,
Abstract
BACKGROUND:
While late gadolinium enhancement (LGE) is proposed as a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC), the potential of LGE to distinguish ARVC from differentials remains unknown. We aimed to assess the diagnostic value of LGE for ARVC diagnosis.
METHODS:
We included 132 subjects (60% male, 47±11 years) who had undergone cardiac magnetic resonance imaging with LGE assessment present for ARVC or ARVC-differentials. ARVC was diagnosed as per 2010 Task Force Criteria (n=55). ARVC-differentials consisted of familial/genetic dilated cardiomyopathy (n=25), myocarditis (n=13), sarcoidosis (n=20), and amyloidosis (n=19). The diagnosis of all differentials was based upon the most current golden standard. Presence of LGE was evaluated using a 7-segment RV and 17-segment LV model. Subsequently, we assessed LGE patterns for every patient individually for fulfilling LV- and/or RV-LGE per Padua criteria, independent of their clinical diagnosis (i.e. phenotype). Diagnostic values were analysed using sensitivity and specificity for any RV-LGE, any LV-LGE, RV-LGE per Padua criteria, and prevalence graphs for LV-LGE per Padua criteria. Optimal integration of LGE for ARVC diagnosis was determined using classification-and-regression-tree analysis.
RESULTS:
one-third (38%) of ARVC patients had RV-LGE, while half (51%) had LV-LGE. RV-LGE was less frequently observed in ARVC vs. non-ARVC patients (38% vs. 58%, p=0.034) leading to a poor discriminatory potential (any RV-LGE: sensitivity 38%, specificity 42%; RV-LGE per Padua criteria: sensitivity 36%, specificity 44%). Compared to ARVC patients, non-ARVC patients more often had LV-LGE (91% vs. 51%, p
CONCLUSIONS:
LGE is often present in ARVC differentials and may lead to false positive diagnoses when used without knowledge of LGE patterns. Moderate RV-LGE without anteroseptal and mid-myocardial LV-LGE is typically observed in ARVC.
Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.
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Phenotypes in Pulmonary Hypertension.
Eur Respir J2024 Jul;():. doi: 2301633.
Weatherald Jason, Hemnes Anna R, Maron Bradley A, Mielniczuk Lisa M, Gerges Christian, Price Laura C, Hoeper Marius M, Humbert Marc,
Abstract
The clinical classification of pulmonary hypertension (PH) has guided diagnosis and treatment of patients with PH for several decades. Discoveries relating to underlying mechanisms, pathobiology, and responses to treatments for PH have informed the evolution in this clinical classification to describe the heterogeneity in PH phenotypes. In more recent years, advances in imaging, computational science, and multi-omic approaches have yielded new insights into potential phenotypes and sub-phenotypes within the existing clinical classification. Identification of novel phenotypes in pulmonary arterial hypertension (PAH) with unique molecular profiles, for example, could lead to new precision therapies. Recent phenotyping studies have also identified groups of patients with PAH that more closely resemble patients with left heart disease (group 2 PH) and lung disease (group 3 PH), which has important prognostic and therapeutic implications. Within group 2 and group 3 PH, novel phenotypes have emerged that reflect a persistent and severe pulmonary vasculopathy that is associated with worse prognosis but still distinct from PAH. In group 4 PH (chronic thromboembolic pulmonary disease) and sarcoidosis (group 5 PH) the current approach to patient phenotyping integrates clinical, hemodynamic and imaging characteristics to guide treatment but applications of multi-omic approaches to sub-phenotyping in these areas are sparse. The next iteration of the PH clinical classification is likely to reflect several emerging PH phenotypes and improve the next generation of prognostication tools, clinical trial design, and improve treatment selection in clinical practice.
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