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Pubblicazioni recenti - cardiac sarcoidosis
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Peripheral blood natural killer cells in sarcoidosis are associated with early cardiac involvement.
Eur J Clin Invest2022 Jan;():e13742. doi: 10.1111/eci.13742.
Vakrakou Aigli G, Kolilekas Lykourgos, Lama Niki, Katsanos Spiros, Stratakos Grigorios, Tsougos Ilias, Manali Effrosyni, Grigoriou Eirini, Psarra Katherina, Kilidireas Constantinos, Papiris Spiros, Kelekis Nikolaos L, Gialafos Elias J,
Abstract
AIM:
To evaluate the distribution of circulating immune cell subsets in peripheral blood of patients with sarcoidosis and investigate if there is an association with an underlying cardiac involvement.
METHODS AND RESULTS:
Eighty-five newly diagnosed treatment-naïve patients with sarcoidosis (50 women) were included in the study. All patients underwent a thorough cardiac investigation, including cardiac magnetic resonance imaging (CMR). Of all patients, 19 (23.53%) had myocardial involvement, and the NK subpopulation in these patients in peripheral blood was significantly decreased compared to patients without (n = 63, p = 0.001 and p = 0.003 respectively). The absolute number of NKT cells (CD3+CD16/56 ) in patients with cardiac involvement was highly correlated with T2 map increased values in MRI (r = -686, p = 0.041) showing that low NKT cell count correlates with the inflammatory process of the heart. No difference in CD19, CD3, CD4, CD8 and CD3 NK cell counts was found between groups. Lung severity was not found to correlate with the number of NK cells.
CONCLUSION:
We found that low NK cell count in peripheral blood of patients with sarcoidosis is associated with cardiac involvement, and the number of NK-T cells correlates with CMR findings indicative of myocardial inflammation. This finding might have a potential clinical application in detecting clinically silent cardiac involvement in sarcoidosis and may also suggest potential targets for therapeutic interventions.
© 2022 Stichting European Society for Clinical Investigation Journal Foundation. Published by John Wiley & Sons Ltd.
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Outcomes after heart transplantation in patients with cardiac sarcoidosis.
ESC Heart Fail2022 Jan;():. doi: 10.1002/ehf2.13789.
Asleh Rabea, Briasoulis Alexandros, Doulamis Ilias, Alnsasra Hilmi, Tzani Aspasia, Alvarez Paulino, Kuno Toshiki, Kampaktsis Polydoros, Kushwaha Sudhir,
Abstract
BACKGROUND:
The number of patients with sarcoidosis requiring heart transplantation (HT) is increasing. The aim of this study was to evaluate outcomes of isolated HT in patients with sarcoid cardiomyopathy and compare them to recipients with non-ischaemic restrictive or dilated cardiomyopathy.
METHODS AND RESULTS:
Adult HT recipients were identified in the UNOS Registry between 1990 and 2020. Patients were grouped according to diagnosis. The cumulative incidences for the all-cause mortality and rejection were compared using Fine and Gray model analysis, accounting for re-transplantation as a competing risk. Rejection was evaluated using logistic regression analysis. We also reviewed characteristics and outcomes of all HT recipients with previous diagnosis of sarcoid cardiomyopathy from a single centre. A total of 30 160 HT recipients were included in the present study (n = 239 sarcoidosis, n = 1411 non-ischaemic restrictive cardiomyopathy, and n = 28 510 non-ischaemic dilated cardiomyopathy). During a total of 194 733 patient-years, all-cause mortality at the latest follow-up was not significantly different when comparing sarcoidosis to non-ischaemic dilated cardiomyopathy [adjusted subhazard ratio (aSHR) 1.46, 95% confidence intervals (CIs): 0.9-2.4, P = 0.12] or restrictive cardiomyopathy (aSHR 1.12, 95% CI: 0.65-1.95, P = 0.67). Accordingly, multivariable analysis suggested that 1 year mortality was not significantly different between sarcoidosis and non-ischaemic dilated cardiomyopathy (aSHR 1.56, 95% CI: 0.9-2.7, P = 0.12) or restrictive cardiomyopathy (aSHR 1.15, 95% CI: 0.61-2.18, P = 0.66). No differences were observed regarding 30 day mortality, treated and hospitalized acute rejection, and 30 day death from graft failure after HT. Thirty-day mortality did not improve significantly in more recent HT eras whereas there was a trend towards improved 1 year mortality in the latest HT era (P = 0.06). Data from the single-centre case review showed excellent long-term outcomes with sirolimus-based immunosuppression.
CONCLUSIONS:
Short-term and long-term post HT outcomes among patients with sarcoid cardiomyopathy are similar to those with common types of non-ischaemic cardiomyopathy.
© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
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Systemic Evaluation of Cutaneous Sarcoidosis: 15 year Dermatology Experience at UHL.
Clin Exp Dermatol2022 Jan;():. doi: 10.1111/ced.15097.
Byrne Berbie, Goh Alicia, Izham Nor, Porter Emma, Field Sinead,
Abstract
Sarcoidosis is a multisystem granulomatous disease which can affect almost any organ including skin, liver, ocular, cardiac, renal, nervous system, musculoskeletal and endocrine. Systemic evaluation is indicated in all patients diagnosed with cutaneous sarcoidosis as it is associated with asymptomatic systemic disease in 30-40% of patients. Guidelines recommend patients diagnosed with sarcoidosis undergo baseline and surveillance investigations including FBC, renal and liver profile, Vitamin D, serum calcium, ECG, CXR, pulmonary function tests (PFTs) and ophthalmology exam to assess for systemic involvement. Recommendations for surveillance monitoring vary on interval duration but include regular FBC, biochemistry, CXR, PFTS with additional investigations and prompt referral to respective specialties as indicated. A retrospective analysis was conducted to evaluate extracutaneous involvement and systemic evaluation of patients diagnosed with cutaneous sarcoidosis from 2014 - 2020. We compared our findings to international guidelines. Of the 18 patients included, cutaneous manifestation was the primary presentation for 67% (n=12). Extracutaneous disease subsequently developed in 67% (n=8) of these patients. Baseline investigations included CXR for 94% (n=17), PFTs 39% (n=7), ECGs 25% (n=4/16), FBC 94% (n=17), renal profile 89% (n=16), LFTs 83% (n=15), serum calcium 89% (n=16) with no vitamin D levels recorded. Specialist referral was required for 89% (n=16), including 94% (n=15) respiratory, 69% (n=11) ophthalmology, and 19% (n=3) nephrology. Results highlighted the importance of a structured protocol for the systemic evaluation of patients diagnosed with cutaneous sarcoidosis. We subsequently developed a baseline and surveillance protocol for the assessment of extracutaneous disease in patients at University Hospital Limerick.
This article is protected by copyright. All rights reserved.
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Histology of Cardiac Sarcoidosis with Novel Considerations Arranged upon a Pathologic Basis.
J Clin Med2022 Jan;11(1):. doi: 251.
Kato Shu, Sakai Yasuhiro, Okabe Asako, Kawashima Yoshiaki, Kuwahara Kazuhiko, Shiogama Kazuya, Abe Masato, Ito Hiroyasu, Morimoto Shin'ichiro,
Abstract
Sarcoidosis is a rare disease of isolated or diffuse granulomatous inflammation. Although any organs can be affected by sarcoidosis, cardiac sarcoidosis is a fatal disorder, and it is crucial to accurately diagnose it to prevent sudden death due to dysrhythmia. Although endomyocardial biopsy is invasive and has limited sensitivity for identifying granulomas, it is the only modality that yields a definitive diagnosis of cardiac sarcoidosis. It is imperative to develop novel pathological approaches for the precise diagnosis of cardiac sarcoidosis. Here, we aimed to discuss commonly used diagnostic criteria for cardiac sarcoidosis and to summarize useful and novel histopathologic criteria of cardiac sarcoidosis. While classical histologic observations including noncaseating granulomas and multinucleated giant cells (typically Langhans type) are the most important findings, others such as microgranulomas, CD68 CD163 pro-inflammatory (M1) macrophage accumulation, CD4/CD8 T-cell ratio, components, lymphangiogenesis, confluent fibrosis, and fatty infiltration may help to improve the sensitivity of endomyocardial biopsy for detecting cardiac sarcoidosis. These novel histologic findings are based on the pathology of cardiac sarcoidosis. We also discussed the principal histologic differential diagnoses of cardiac sarcoidosis, such as tuberculosis myocarditis, fungal myocarditis, giant cell myocarditis, and dilated cardiomyopathy.
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Myocarditis in systemic immune-mediated diseases: Prevalence, characteristics and prognosis. A systematic review.
Autoimmun Rev2022 Jan;21(4):103037. doi: S1568-9972(22)00007-6.
Cheng Chun-Yan, Baritussio Anna, Giordani Andrea Silvio, Iliceto Sabino, Marcolongo Renzo, Caforio Alida L P,
Abstract
Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic, granulomatous, and clinical features, ranging from a completely asymptomatic patient to life-threatening cardiogenic shock or arrhythmias. Myocarditis can be part of some SIDs, such as sarcoidosis, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, dermato-polymyositis, eosinophilic granulomatosis with polyangiitis and other vasculitis syndromes, but also of some organ-based immune-mediated diseases with systemic expression, such as chronic inflammatory bowel diseases. The aim of this review is to describe the prevalence, main clinical characteristics and prognosis of myocarditis associated with SIDs.
Copyright © 2022. Published by Elsevier B.V.
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Bidirectional Ventricular Tachycardia in a Patient With Fulminant Myocarditis Secondary to Cardiac Sarcoidosis Mimicking Giant Cell Myocarditis.
CJC Open2021 Dec;3(12):1509-1512. doi: 10.1016/j.cjco.2021.07.007.
Durocher Daniel, El-Hajjaji Imane, Gilani Syed O, Leong-Sit Peter, Davey Ryan A, De Sabe K,
Abstract
Differentiating between sarcoidosis and giant cell myocarditis (GCM) based on clinical presentation is difficult. We present the case of a 57-year-old woman who was initially diagnosed with GCM based on endomyocardial biopsy. The patient was refractory to standard management for GCM and went on to develop bidirectional ventricular tachycardia, a finding suggestive of sarcoidosis. Unfortunately, the patient eventually needed cardiac transplantation. The explanted heart demonstrated cardiac sarcoidosis. Bidirectional ventricular tachycardia has not been demonstrated in GCM, and its presence may help in distinguishing between GCM and cardiac sarcoidosis.
© 2021 The Authors.
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Cardiac sarcoidosis involving the papillary muscle: A case report.
HeartRhythm Case Rep2021 Dec;7(12):801-805. doi: 10.1016/j.hrcr.2021.08.014.
Ando Victoria, Koestner Simon, Pruvot Etienne, Kamani Christel-Hermann, Ganiere Vincent,
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Risk and Predictors of Mortality After Implantable Cardioverter-Defibrillator Implantation in Patients with Sarcoid Cardiomyopathy.
Am Heart J2021 Dec;():. doi: S0002-8703(21)00491-9.
Higgins Angela Y, Annapureddy Amarnath R, Wang Yongfei, Minges Karl E, Bellumkonda Lavanya, Lampert Rachel, Rosenfeld Lynda E, Jacoby Daniel L, Curtis Jeptha P, Miller Edward J, Freeman James V,
Abstract
BACKGROUND:
Implantable cardioverter-defibrillators (ICDs) are recommended for patients with cardiac sarcoidosis (CS) with an indication for pacing, prior ventricular arrhythmias, cardiac arrest, or LVEF
METHODS:
Using data from the NCDR ICD Registry between April 1, 2010 and December 31, 2015, we evaluated a propensity matched cohort of CS patients implanted with ICDs versus non-ischemic cardiomyopathies (NICM). We compared mortality using Kaplan-Meier survival curves and Cox proportional hazards models.
RESULTS:
We identified 1,638 patients with CS and 8,190 propensity matched patients with NICM. The rate of death at one and two years was similar in patients with CS and patients with NICM (5.2% vs 5.4%, p 0.75 and 9.0% vs 9.3%, p 0.72, respectively). After adjusting for other covariates, patients with CS had similar mortality at two years after ICD implantations compared with NICM patients (RR 1.03, 95% CI 0.87-1.23). Among patients with CS, multivariable logistic regression identified 6 factors significantly associated with increased two-year mortality: presence of heart failure (HR1.92, 95% CI 1.44-3.22), New York Heart Association (NYHA) Class III heart failure (HR 1.68, 95% CI 1.16-2.45), NYHA Class IV heart failure (HR 3.08, 95% CI 1.49-6.39), atrial fibrillation/flutter (HR 1.66, 95% CI 1.17-2.35), chronic lung disease (HR 1.64, 95% CI 1.17-2.29), creatinine >2.0 mg/dL (HR 4.07, 95% CI 2.63-6.30), and paced rhythm (HR 2.66, 95% CI 1.07-6.59).
CONCLUSION:
Mortality following ICD implantation was similar in CS patients compared with propensity matched NICM patients. Presence of heart failure, NYHA class, atrial fibrillation/flutter, chronic lung disease, renal dysfunction, and paced rhythm at time of implantation were all predictors of increased two-year mortality among CS patients with ICDs.
Copyright © 2021. Published by Elsevier Inc.
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Inflammatory cardiomyopathy of possibly overlapping aetiology: a case posing treatment dilemma and potential association.
ESC Heart Fail2021 Dec;():. doi: 10.1002/ehf2.13771.
Nakagama Shun, Candray Katherine, Yamamoto Tasuku, Tsugeno Yuta, Nakagama Yu, Kido Yasutoshi, Nitahara Yuko, Maejima Yasuhiro, Sasano Tetsuo,
Abstract
We report on a 52-year-old Brazilian immigrant woman with past histories of chronic kidney disease and uveitis, presenting with symptomatic atrioventricular block. Her country of origin being endemic for Trypanosoma cruzi infection, we suspected Chagas disease as the aetiology, diagnosis of which was confirmed by serological tests. Further systemic workup identified an emerging nodular lesion in the lung, which turned out to be a sarcoid epithelioid granuloma on biopsy. Involvement of the kidneys and eyes was suggestive of systemic extension of the lung sarcoidosis. Although imaging modalities did not detect inflammatory foci in the myocardium, the rare coexistence of histologically proven sarcoidosis raised the intriguing concept of cardiac manifestation having arisen from two possibly overlapping aetiologies: Chagas disease and cardiac sarcoidosis. The case highlights a treatment dilemma increasingly likely to be encountered in this globalized world, and also raises the potential, but intriguing, association of these two diseases.
© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
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PET Imaging in Cardiac Sarcoidosis: A Narrative Review with Focus on Novel PET Tracers.
Pharmaceuticals (Basel)2021 Dec;14(12):. doi: 1286.
Saric Petar, Young Kathleen A, Rodriguez-Porcel Martin, Chareonthaitawee Panithaya,
Abstract
Sarcoidosis is a multi-system inflammatory disease characterized by the development of inflammation and noncaseating granulomas that can involve nearly every organ system, with a predilection for the pulmonary system. Cardiac involvement of sarcoidosis (CS) occurs in up to 70% of cases, and accounts for a significant share of sarcoid-related mortality. The clinical presentation of CS can range from absence of symptoms to conduction abnormalities, heart failure, arrhythmias, valvular disease, and sudden cardiac death. Given the significant morbidity and mortality associated with CS, timely diagnosis is important. Traditional imaging modalities and histologic evaluation by endomyocardial biopsy often provide a low diagnostic yield. Cardiac positron emission tomography (PET) has emerged as a leading advanced imaging modality for the diagnosis and management of CS. This review article will summarize several aspects of the current use of PET in CS, including indications for use, patient preparation, image acquisition and interpretation, diagnostic and prognostic performance, and evaluation of treatment response. Additionally, this review will discuss novel PET radiotracers currently under study or of potential interest in CS.
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Cardiac Sarcoidosis: When and How to Treat Inflammation.
Card Fail Rev2021 Mar;7():e17. doi: 10.15420/cfr.2021.16.
Giblin Gerard T, Murphy Laura, Stewart Garrick C, Desai Akshay S, Di Carli Marcelo F, Blankstein Ron, Givertz Michael M, Tedrow Usha B, Sauer William H, Hunninghake Gary M, Dellaripa Paul F, Divakaran Sanjay, Lakdawala Neal K,
Abstract
Sarcoidosis is a complex, multisystem inflammatory disease with a heterogeneous clinical spectrum. Approximately 25% of patients with systemic sarcoidosis will have cardiac involvement that portends a poorer outcome. The diagnosis, particularly of isolated cardiac sarcoidosis, can be challenging. A paucity of randomised data exist on who, when and how to treat myocardial inflammation in cardiac sarcoidosis. Despite this, corticosteroids continue to be the mainstay of therapy for the inflammatory phase, with an evolving role for steroid-sparing and biological agents. This review explores the immunopathogenesis of inflammation in sarcoidosis, current evidence-based treatment indications and commonly used immunosuppression agents. It explores a multidisciplinary treatment and monitoring approach to myocardial inflammation and outlines current gaps in our understanding of this condition, emerging research and future directions in this field.
Copyright © 2021, Radcliffe Cardiology.
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Atrial fibrillation as a presenting symptom of Cardiac Sarcoid.
J Atr Fibrillation;14(1):20200484. doi: 10.4022/jafib.20200484.
Hussain Ali, C Yiu Alvin, A Okonkwo Uzoagu, O'shea John-Paul,
Abstract
We submit an unusual presentation of spontaneous atrial fibrillation in a young fit active-duty U.S. military African-American male without evidence of structural heart disease. His atrial fibrillation was refractory to several ablation treatments over the course of 3 years. Subsequently he was diagnosed with extracardiac sarcoidosis and fluorodeoxyglucose-positron emission tomography (FDG-PET) scan identified bi-atrial hypermetabolic lesions, concerning for cardiac sarcoidosis. Given the low incidence of atrial fibrillation in patients
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Advances in Diagnostic Imaging for Cardiac Sarcoidosis.
J Clin Med2021 Dec;10(24):. doi: 5808.
Manabe Osamu, Oyama-Manabe Noriko, Aikawa Tadao, Tsuneta Satonori, Tamaki Nagara,
Abstract
Sarcoidosis is a systemic granulomatous disease of unknown etiology, and its clinical presentation depends on the affected organ. Cardiac sarcoidosis (CS) is one of the leading causes of death among patients with sarcoidosis. The clinical manifestations of CS are heterogeneous, and range from asymptomatic to life-threatening arrhythmias and progressive heart failure due to the extent and location of granulomatous inflammation in the myocardium. Advances in imaging techniques have played a pivotal role in the evaluation of CS because histological diagnoses obtained by myocardial biopsy tend to have lower sensitivity. The diagnosis of CS is challenging, and several approaches, notably those using positron emission tomography and cardiac magnetic resonance imaging (MRI), have been reported. Delayed-enhanced computed tomography (CT) may also be used for diagnosing CS in patients with MRI-incompatible devices and allows acceptable evaluation of myocardial hyperenhancement in such patients. This article reviews the advances in imaging techniques for the evaluation of CS.
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The usefulness of repeated CMR and FDG PET/CT in the diagnosis of patients with initial possible cardiac sarcoidosis.
EJNMMI Res2021 Dec;11(1):129. doi: 10.1186/s13550-021-00870-y.
Mathijssen H, Tjoeng T W H, Keijsers R G M, Bakker A L M, Akdim F, van Es H W, van Beek F T, Veltkamp M V, Grutters J C, Post M C,
Abstract
BACKGROUND:
Cardiac sarcoidosis (CS) diagnosis is usually based on advanced imaging techniques and multidisciplinary evaluation. Diagnosis is classified as definite, probable, possible or unlikely. If diagnostic confidence remains uncertain, cardiac imaging can be repeated. The objective is to evaluate the usefulness of repeated cardiac magnetic resonance imaging (CMR) and fluorodeoxyglucose positron emission tomography (FDG PET/CT) for CS diagnosis in patients with an initial "possible" CS diagnosis.
METHODS:
We performed a retrospective cohort study in 35 patients diagnosed with possible CS by our multidisciplinary team (MDT), who received repeated CMR and FDG PET/CT within 12 months after diagnosis. Imaging modalities were scored on abnormalities suggestive for CS and classified as CMR+/PET+, CMR+/PET-, CMR-/PET+ and CMR-/PET-. Primary endpoint was final MDT diagnosis of CS.
RESULTS:
After re-evaluation, nine patients (25.7%) were reclassified as probable CS and 16 patients (45.7%) as unlikely CS. Two patients started immunosuppressive treatment after re-evaluation. At baseline, eleven patients (31.4%) showed late gadolinium enhancement (LGE) on CMR (CMR+) and 26 (74.3%) patients showed myocardial FDG-uptake (PET+). At re-evaluation, nine patients (25.7%) showed LGE (CMR+), while 16 patients (45.7%) showed myocardial FDG-uptake (PET+). When considering both imaging modalities together, 82.6% of patients with CMR-/PET+ at baseline were reclassified as possible or unlikely CS, while 36.4% of patients with CMR+ at baseline were reclassified as probable CS. Three patients with initial CMR-/PET+ showed LGE at re-evaluation.
CONCLUSION:
Repeated CMR and FDG PET/CT may be useful in establishing or rejecting CS diagnosis, when initial diagnosis is uncertain. However, clinical relevance has to be further determined.
© 2021. The Author(s).
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Stable Extent of Recurrently Active Cardiac and Cutaneous Sarcoidosis.
Front Med (Lausanne)2021 ;8():729229. doi: 10.3389/fmed.2021.729229.
Patterson Karen C, Rosenbach Misha, Bravo Paco E, Dubroff Jacob G,
Abstract
Recurrent or persistently active sarcoidosis is a risk factor for permanent organ damage. Whether this damage is due to accumulated focal injuries or progressive disease extent is not known, as the natural history of chronic inflammation in sarcoidosis is poorly characterized. The objective of this study is to determine the pattern of disease in recurrently active sarcoidosis. We identified patients with recurrent cardiac sarcoidosis ( = 21) retrospectively from an imaging database, and with recurrent cutaneous sarcoidosis ( = 17) from a prospective registry. The longitudinal patterns of cardiac sarcoidosis were established by findings on cardiac positron emission tomography scans, and of cutaneous sarcoidosis by the validated Cutaneous Sarcoidosis Activity and Morphology Instrument clinical scoring system. Patterns of recurrent disease were compared to baseline findings. Recurrent sarcoidosis occurred in a nearly identical pattern and distribution as baseline disease, and spread of disease was rarely observed for both cardiac and cutaneous sarcoidosis: 97% of heart segments positive on recurrence scans were positive on baseline scans, and only one new region of facial disease was observed. In some cases, recurrence followed years of apparent remission. Across phenotypes, and across a long period of follow-up, the extent of sarcoidosis was stable in spite of fluctuations in disease activity. For patients with a demonstrated history of recurrent disease affecting critical organs, our findings support the need for long-term follow-up.
Copyright © 2021 Patterson, Rosenbach, Bravo and Dubroff.
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Comparison of heart rates at fixed percentages and the ventilatory thresholds in patients with interstitial lung disease.
Scand J Med Sci Sports2021 Dec;():. doi: 10.1111/sms.14117.
Vonbank Karin, Lehmann Antje, Bernitzky Dominik, Gysan Maximilian Robert, Simon Stefan, Krotka Pavla, Zwick Ralf-Harun, Idzko Marco, Burtscher Martin,
Abstract
Heart rate (HR) responses to maximal exercise are commonly used for the prescription of training intensities in pulmonary rehabilitation. Those intensities are usually based on fixed percentages of peak HR (HRpeak), heart rate reserve (HRR), or peak work load (Wpeak), and rarely on HRs at the individual ventilatory thresholds (VT1 and VT2) derived from cardiopulmonary exercise testing (CPET). For patients suffering from interstitial lung disease (ILD), data on cardiorespiratory responses to CPET are scarce. Thus, the aim of this study was to record cardiorespiratory responses to CPET and to compare fixed HR percentages with HRs at VT1 and VT2 in ILD patients. A total of 120 subjects, 80 ILD patients and 40 healthy controls, underwent a symptom-limited CPET. From the ILD patient, 32 suffered from idiopathic pulmonary fibrosis (IPF), 37 from connective tissue disease (CTD), and 11 from sarcoidosis. HRs at fixed percentages, that is, at 70%HRpeak, at 70%Wpeak, and at 60%HRR were significantly lower in the ILD patients compared with the control group (p-values: 0.001, 0.044, and 0.011). Large percentages of HR values at 70%Wpeak and 60%HRR ranged between the HRs at VT1 and VT2 in ILD subgroups and controls as well. HRs at 70%HRpeak were lower than HRs at VT1 in 66% of the IPF patients, 54% of the CTD patients, and 55% of patients with sarcoidosis compared with 18% in the control group. Our findings demonstrate a considerable scattering of fixed HR percentages compared with HRs at the individual VTs derived from CPET in ILD patients. These findings may provide valuable information for the prescription of exercise intensity in pulmonary rehabilitation of ILD patients.
© 2021 The Authors. Scandinavian Journal of Medicine & Science In Sports published by John Wiley & Sons Ltd.
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How to risk-stratify cardiac sarcoidosis patients with normal or near-normal ventricular function?
Heart Rhythm -
Fulminant cardiac and renal sarcoidosis revealed by electron microscope: challenging aspect of diagnosis.
Eur Heart J Case Rep2021 Nov;5(11):ytab298. doi: 10.1093/ehjcr/ytab298.
Naito Seiichiro, Tsujinaga Shingo, Kamiya Kiwamu, Nagai Toshiyuki, Anzai Toshihisa,
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Combined simultaneous FDG-PET/MRI with T1 and T2 mapping as an imaging biomarker for the diagnosis and prognosis of suspected cardiac sarcoidosis.
Eur J Hybrid Imaging2021 Dec;5(1):24. doi: 10.1186/s41824-021-00119-w.
Cheung Edward, Ahmad Sarah, Aitken Matthew, Chan Rosanna, Iwanochko Robert M, Balter Meyer, Metser Ur, Veit-Haibach Patrick, Billia Filio, Moayedi Yasbanoo, Ross Heather J, Hanneman Kate,
Abstract
PURPOSE:
To evaluate the diagnostic and prognostic significance of combined cardiac F-fluorodeoxyglucose (FDG) PET/MRI with T1/T2 mapping in the evaluation of suspected cardiac sarcoidosis.
METHODS:
Patients with suspected cardiac sarcoidosis were prospectively enrolled for cardiac F-FDG PET/MRI, including late gadolinium enhancement (LGE) and T1/T2 mapping with calculation of extracellular volume (ECV). The final diagnosis of cardiac sarcoidosis was established using modified JMHW guidelines. Major adverse cardiac events (MACE) were assessed as a composite of cardiovascular death, ventricular tachyarrhythmia, bradyarrhythmia, cardiac transplantation or heart failure. Statistical analysis included Cox proportional hazard models.
RESULTS:
Forty-two patients (53?±?13 years, 67% male) were evaluated, 13 (31%) with a final diagnosis of cardiac sarcoidosis. Among patients with cardiac sarcoidosis, 100% of patients had at least one abnormality on PET/MRI: FDG uptake in 69%, LGE in 100%, elevated T1 and ECV in 100%, and elevated T2 in 46%. FDG uptake co-localized with LGE in 69% of patients with cardiac sarcoidosis compared to 24% of those without, p?=?0.014. Diagnostic specificity for cardiac sarcoidosis was highest for FDG uptake (69%), elevated T2 (79%), and FDG uptake co-localizing with LGE (76%). Diagnostic sensitivity was highest for LGE, elevated T1 and ECV (100%). After median follow-up duration of 634 days, 13 patients experienced MACE. All patients who experienced MACE had LGE, elevated T1 and elevated ECV. FDG uptake (HR 14.7, p?=?0.002), elevated T2 (HR 9.0, p?=?0.002) and native T1 (HR 1.1 per 10 ms increase, p?=?0.044) were significant predictors of MACE even after adjusting for left ventricular ejection fraction and immune suppression treatment. The presence of FDG uptake co-localizing with LGE had the highest diagnostic performance overall (AUC 0.73) and was the best predictor of MACE based on model goodness of fit (HR 14.9, p?=?0.001).
CONCLUSIONS:
Combined cardiac FDG-PET/MRI with T1/T2 mapping provides complementary diagnostic information and predicts MACE in patients with suspected cardiac sarcoidosis.
© 2021. The Author(s).
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Utility of electrophysiologic testing for sudden death risk stratification in cardiac sarcoidosis patients with mildly impaired left ventricular function.
Respir Med2021 Dec;191():106712. doi: S0954-6111(21)00420-0.
Tandon Pranai, Mosleh Tayseer, Mustafa Ali, Miodownik Hope, Miller Marc, Morgenthau Adam S,
Abstract
BACKGROUND:
Ventricular arrhythmias (VA) account for at least 25% of deaths caused by cardiac sarcoidosis (CS) and may arise in patients with mildly impaired LVEF (>35%).
OBJECTIVE:
In the current study, we examine whether EP study may be used for sudden death risk stratification in CS patients who have mildly impaired LVEF and a diagnosis of highly probable or probable CS according to the World Association of Sarcoidosis and Other Granulomatous Diseases Sarcoidosis Organ Assessment Instrument (WASOGI).
METHODS:
All patients: (1) exhibited a diagnosis of highly probable or probable CS according to the WASOGI, (2) exhibited cardiac MRI findings consistent with CS, (3) exhibited LVEF >45% and (4) underwent EP study. Device interrogations, transmissions and medical records were reviewed for all patients.
RESULTS:
We identified 46 CS patients with mildly impaired LVEF. VA were induced in 11 patients and 10/11 patients underwent ICD placement. Thirty-five patients had no VA and 24/35 patients underwent placement of an ILR. During the follow-up period, the VA event rate was 6.5%. The negative and positive predictive values of the EP study for the development of VA were 100% and 27.2%, respectively.
CONCLUSIONS:
In CS patients with mildly impaired LVEF and a diagnosis of highly probable or probable CS, a negative EP study was highly predictive of the absence of VA. The successful execution of future prospective studies is contingent upon enrollment of phenotypically homogenous cardiac sarcoidosis patients.
Copyright © 2021 Elsevier Ltd. All rights reserved.
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