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Pubblicazioni recenti - cardiac sarcoidosis
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A case of cardiac sarcoidosis with left ventricular thrombus formation after administration of prednisolone.
J Med Ultrason (2001)2019 Nov;():. doi: 10.1007/s10396-019-00985-9.
Akasaka Toshihiko, Sugihara Shinobu, Kinugasa Yoshiharu, Kato Masahiko, Yamamoto Kazuhiro,
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Detailed visualization of the right and left ventricular, left atrial, and epicardial involvement of cardiac sarcoidosis with novel semiconductor PET/CT.
Eur J Nucl Med Mol Imaging2019 Nov;():. doi: 10.1007/s00259-019-04577-0.
Manabe Osamu, Oyama-Manabe Noriko, Nagai Toshiyuki, Furuya Sho, Anzai Toshihisa,
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Results of the standard set for pulmonary sarcoidosis: feasibility and multicentre outcomes.
ERJ Open Res2019 Oct;5(4):. doi: 00094-2019.
Kampstra Nynke A, van der Nat Paul B, Dijksman Lea M, van Beek Frouke T, Culver Daniel A, Baughman Robert P, Renzoni Elisabetta A, Wuyts Wim, Kouranos Vasilis, Zanen Pieter, Wijsenbeek Marlies S, Eijkemans Marinus J C, Biesma Douwe H, van der Wees Philip J, Grutters Jan C,
Abstract
Our study presents findings on a previously developed standard set of clinical outcome data for pulmonary sarcoidosis patients. We aimed to assess whether changes in outcome varied between the different centres and to evaluate the feasibility of collecting the standard set retrospectively. This retrospective observational comparative benchmark study included six interstitial lung disease expert centres based in the Netherlands, Belgium, the UK and the USA. The standard set of outcome measures included 1) mortality, 2) changes in pulmonary function (forced vital capacity (FVC), forced expiratory volume in 1?s, diffusing capacity of the lung for carbon monoxide), 3) soluble interleukin-2 receptor (sIL-2R) change, 4) weight changes, 5) quality-of-life (QoL) measures, 6) osteoporosis and 7) clinical outcome status (COS). Data collection was considered feasible if the data were collected in ?80% of all patients. 509 patients were included in the retrospective cohort. In total six patients died, with a mean survival of 38±23.4?months after the diagnosis. Centres varied in mean baseline FVC, ranging from 110 (95% CI 92-124)% predicted to 99 (95% CI 97-123)% pred. Mean baseline body mass index (BMI) of patients in the different centres varied between 27 (95% CI 23.6-29.4)?kg·m and 31.8 (95% CI 28.1-35.6) kg·m. 310 (60.9%) patients were still on systemic therapy 2?years after the diagnosis. It was feasible to measure mortality, changes in pulmonary function, weight changes and COS. It is not (yet) feasible to retrospectively collect sIL-2R, osteoporosis and QoL data internationally. This study shows that data collection for the standard set of outcome measures for pulmonary sarcoidosis was feasible for four out of seven outcome measures. Trends in pulmonary function and BMI were similar for different hospitals when comparing different practices.
Copyright ©ERS 2019.
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2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: Executive summary.
Heart Rhythm2019 Nov;16(11):e373-e407. doi: S1547-5271(19)30852-5.
Towbin Jeffrey A, McKenna William J, Abrams Dominic J, Ackerman Michael J, Calkins Hugh, Darrieux Francisco C C, Daubert James P, de Chillou Christian, DePasquale Eugene C, Desai Milind Y, Estes N A Mark, Hua Wei, Indik Julia H, Ingles Jodie, James Cynthia A, John Roy M, Judge Daniel P, Keegan Roberto, Krahn Andrew D, Link Mark S, Marcus Frank I, McLeod Christopher J, Mestroni Luisa, Priori Silvia G, Saffitz Jeffrey E, Sanatani Shubhayan, Shimizu Wataru, van Tintelen J Peter, Wilde Arthur A M, Zareba Wojciech,
Abstract
Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
Copyright © 2019 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.
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Blue Toes at High Altitude: Peripheral Cyanosis.
Am J Med2019 Oct;():. doi: S0002-9343(19)30878-2.
Gupta Amol, Gupta Ravi, Kumar Vinod, Samarany Samir,
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Natural evolution of cardiac sarcoidosis in an asymptomatic patient: a case report.
Eur Heart J Case Rep2019 Sep;3(3):ytz099. doi: 10.1093/ehjcr/ytz099.
Degtiarova Ganna, Gheysens Olivier, Van Cleemput Johan, Wuyts Wim, Bogaert Jan,
Abstract
Background:
Sarcoidosis is a multi-organ granulomatous disease of unknown aetiology. Adverse outcome related with cardiac involvement, makes early diagnosis of cardiac sarcoidosis crucial.
Case summary:
In a 55-year-old man presenting with recurrent pulmonary infections, computed tomography (CT) showed several enlarged mediastinal lymph nodes and no lung pathology. Subsequent mediastinoscopy revealed the diagnosis of sarcoidosis. Further screening for organ involvement showed multifocal cardiac involvement both on cardiac magnetic resonance (CMR) and 18-F-fluorodeoxyglucose-positron emission tomography-computed tomography (F-FDG PET-CT). Because of the lack of functional deterioration and clinical symptoms, no steroid treatment was initiated and regular follow-up of cardiac abnormalities was performed by CMR. Unremarkable progression of cardiac involvement during the first 2?years of follow-up turned into a dramatic involvement after 4?years, with the increase in the number and size of lesions at late gadolinium enhancement (LGE) CMR. Late gadolinium enhancement areas matched the regions of strongly increased F-FDG uptake. For the first time, the patient started complaining on shortness of breath, electrocardiography showed an atrioventricular block Grade 1. Cardiac biomarkers and cardiac function were still preserved. Steroid treatment was started. Although an electrophysiology study was negative, Holter monitoring showed ventricular arrhythmia. Cardioverter-defibrillator was implanted.
Discussion:
This case shows the progression of cardiac sarcoidosis on CMR in an asymptomatic untreated patient over a 4-year period, and rises the awareness of possible severe cardiac damage even in the absence of clinical signs of cardiac involvement. Combination of PET and CMR is appealing to better understand the evolution of cardiac sarcoidosis and may help in the management of such patients.
© The Author(s) 2019. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Endobronchial ultrasound-guided intranodal forceps biopsy (EBUS-IFB)-technical review.
J Thorac Dis2019 Sep;11(9):4049-4058. doi: 10.21037/jtd.2019.08.106.
Cheng George, Mahajan Amit, Oh Scott, Benzaquen Sadia, Chen Alexander,
Abstract
Endobronchial ultrasound (EBUS) and transbronchial needle aspiration (TBNA) have changed the landscape of pulmonology. Mediastinal structures beyond the confines of airway walls are visualized in real-time with EBUS, leading to improved accuracy of tissue sampling and diagnostic yield. With the development of various needle sizes ranging from 25-G to 19-G, the sampling of lymph nodes is becoming easier and more commonplace. Yet, certain conditions such as sarcoidosis and lymphoma may still be difficult to diagnose via EBUS-TBNA. Furthermore, in the age of targeted therapy, there are more demands on EBUS-TBNA samples for molecular marker testing and next-generation sequencing. Here, we present a complementary methodology, EBUS-guided intranodal forceps biopsy (EBUS-IFB), for tissue acquisition that may help address these deficiencies. Specifically, we aim to propose indications, contraindications, outline approaches in performing IFB, and provide an overview of the data for this complementary technique.
2019 Journal of Thoracic Disease. All rights reserved.
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Postural Conversion Computed Tomography for the Diagnosis of Pneumopericardium due to Perforation by the Active Atrial Lead: A Case Report.
Intern Med2019 Oct;():. doi: 10.2169/internalmedicine.3729-19.
Futami Makito, Komaki Tomo, Arinaga Toyonori, Morii Joji, Sugihara Makoto, Ogawa Masahiro, Miura Shin-Ichiro,
Abstract
A 71-year-old woman with cardiac sarcoidosis underwent an implantable cardioverter-defibrillator implantation in the left precordium to prevent fatal arrhythmias. Two weeks later, she presented with dyspnea. Chest X-ray revealed right pneumothorax due to the active atrial lead perforation. Subsequently, air was detected surrounding the heart. Although it was difficult to differentiate pneumopericardium from pneumomediastinum, postural conversion computed tomography (CT) in the supine and prone positions documented air migration in the pericardial cavity and diagnosed pneumopericardium. This rare case of pneumopericardium combined with pneumothorax contralateral to the venous access site highlights the utility of postural conversion CT for diagnosis of pneumopericardium.
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Detection of Active Inflammation Status Around Ventricular Aneurysms in Patients With Cardiac Sarcoidosis.
Circ J2019 Oct;():. doi: 10.1253/circj.CJ-19-0248.
Nanno Takuma, Kobayashi Shigeki, Yoshitomi Ryosuke, Fujii Shohei, Kajii Toshiro, Kohno Michiaki, Ishiguchi Hironori, Okuda Shinichi, Okada Munemasa, Suga Kazuyoshi, Yano Masafumi,
Abstract
BACKGROUND:
Little is known about the pattern of isotope accumulation in the heart on F-fluorodeoxyglucose (F-FDG) positron emission tomography in patients with cardiac sarcoidosis (CS) complicated by ventricular aneurysm (VA).Methods?and?Results:We prospectively enrolled 82 consecutive patients with CS; 54 patients with active CS (presence of abnormal F-FDG accumulation in the heart) were subdivided into VA (n=17) and non-VA groups (n=37). Strong F-FDG accumulation surrounding the VA and its disappearance in the VA center was observed in all patients with VA, probably because of scar formation at the VA. Peak standardized uptake value was higher around the VA than in the VA center (5.1±2.1 vs. 2.2±0.6, P=0.0003) and the VA center had no F-FDG accumulation (VA center: 2.2±0.6 vs. control area: 2.1±0.6, P=0.37). On the other hand, in non-VA patients with LV wall thinning (n=28), F-FDG accumulation was significantly high, even in the area of LV wall thinning (LV wall thinning area: 3.1±0.8 vs. control area: 2.0±0.6, P=0.00002).
CONCLUSIONS:
A pattern of strong F-FDG accumulation surrounding the VA and its disappearance in the VA center might be characteristic in patients with CS complicated by VA. Careful attention to FDG uptake would further elucidate CS pathophysiology and aid in the early treatment of VA.
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An Unusual Cause of Sudden Cardiac Death.
West Afr J Med;36(3):286-289.
Bamidele A, Adnan S, Aled P,
Abstract
Sarcoidosis is a chronic granulomatous disease with multi-systemic involvement and with varied forms of presentation. The diagnosis can often be a challenge particularly due to the non-specific nature of the disease. It is often silent and may require no active treatment except during flares. We present the case of a middle age lady with pulmonary sarcoidosis who had sudden death from cardiac affectation without prior cardiac symptoms. We concluded that active cardiac assessment is a prudent step in patients presenting with sarcoidosis regardless of the organ of affectation at presentation or diagnosis.
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The rate of myocardial perfusion recovery after steroid therapy and its implication for cardiac events in cardiac sarcoidosis and primarily preserved left ventricular ejection fraction.
J Nucl Cardiol2019 Oct;():. doi: 10.1007/s12350-019-01916-4.
Koyanagawa Kazuhiro, Naya Masanao, Aikawa Tadao, Manabe Osamu, Furuya Sho, Kuzume Masato, Oyama-Manabe Noriko, Ohira Hiroshi, Tsujino Ichizo, Anzai Toshihisa,
Abstract
BACKGROUND:
Sarcoidosis is a multisystemic disorder of unknown cause characterized by immune granuloma formation in the involved organs. Few studies have reported on the myocardial perfusion changes by immunosuppression therapy in cardiac sarcoidosis (CS). Additionally, the relationship between myocardial perfusion changes and prognosis is unknown. Therefore, this study aimed to clarify myocardial perfusion recovery after steroid therapy and its prognostic value for major adverse cardiac events (MACE) in patients with CS.
METHODS AND RESULTS:
Thirty-eight consecutive patients with CS {median age, 63 [interquartile range (IQR) 51-68] years; 10 men} underwent both F-fluorodeoxyglucose positron emission tomography/computed tomography (CT) and electrocardiography-gated single-photon emission CT (SPECT) pre- and post-steroid therapy. Patients with improved or preserved myocardial perfusion after post-therapy were defined as the recovery group and those with worsened myocardial perfusion as the non-recovery group. Twenty-six patients (68%) were categorized as the recovery group. MACE occurred in eight patients. The Kaplan-Meier curves revealed a significantly higher rate of MACE in the non-recovery group (17.4%/y vs 2.9%/y, P = 0.007).
CONCLUSIONS:
Myocardial perfusion was recovered by steroid therapy in 61% and preserved in 8% of patients. Myocardial perfusion recovery after steroid therapy was significantly associated with a low incidence of MACE.
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JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis?- Digest Version.
Circ J2019 Oct;83(11):2329-2388. doi: 10.1253/circj.CJ-19-0508.
Terasaki Fumio, Azuma Arata, Anzai Toshihisa, Ishizaka Nobukazu, Ishida Yoshio, Isobe Mitsuaki, Inomata Takayuki, Ishibashi-Ueda Hatsue, Eishi Yoshinobu, Kitakaze Masafumi, Kusano Kengo, Sakata Yasushi, Shijubo Noriharu, Tsuchida Akihito, Tsutsui Hiroyuki, Nakajima Takatomo, Nakatani Satoshi, Horii Taiko, Yazaki Yoshikazu, Yamaguchi Etsuro, Yamaguchi Tetsuo, Ide Tomomi, Okamura Hideo, Kato Yasuchika, Goya Masahiko, Sakakibara Mamoru, Soejima Kyoko, Nagai Toshiyuki, Nakamura Hiroshi, Noda Takashi, Hasegawa Takuya, Morita Hideaki, Ohe Tohru, Kihara Yasuki, Saito Yoshihiko, Sugiyama Yukihiko, Morimoto Shin-Ichiro, Yamashina Akira, ,
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[Update of diagnosis and management of cardiac sarcoidosis].
Zhonghua Jie He He Hu Xi Za Zhi2019 Oct;42(10):771-776. doi: 10.3760/cma.j.issn.1001-0939.2019.10.012.
Sun Y X, Li S, Shao C, Huang H, Xu Z J,
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Deterioration of biventricular strain is an early marker of cardiac involvement in confirmed sarcoidosis.
Eur Heart J Cardiovasc Imaging2019 Sep;():. doi: jez235.
Kusunose Kenya, Fujiwara Mika, Yamada Hirotsugu, Nishio Susumu, Saijo Yoshihito, Yamada Nao, Hirata Yukina, Torii Yuta, Ise Takayuki, Yamaguchi Koji, Fukuda Daiju, Yagi Shusuke, Soeki Takeshi, Wakatsuki Tetsuzo, Sata Masataka,
Abstract
AIMS:
Risk assessment of developing cardiac involvement in systemic sarcoidosis can be challenging because of limited data. Recently, attention has been given to left ventricular and right ventricular (LV and RV) involvement in cardiac sarcoidosis (CS) and its prevalence, relevance, and prognostic value. The aim of this study was to assess the role of biventricular strain to predict prognosis in confirmed sarcoidosis patients.
METHODS AND RESULTS:
LV and RV longitudinal strains (LSs) were evaluated by 2D speckle tracking in 139 consecutive confirmed sarcoidosis patients without other pre-existing structural heart diseases, and 52 age- and gender-matched control subjects. The primary endpoint was CS-related events (cardiac death or development of cardiac involvement). Sarcoidosis without cardiac involvement had significantly lower LV and RV free wall LS compared with control subjects. Basal LS had a higher area under the curve for differentiation of sarcoidosis in patients without cardiac involvement compared to control (cut-off value: -18% with 89% sensitivity and 69% specificity). During a median period of 50 months, the occurrence of CS-related events was observed in 20 patients. In a multivariate analysis, basal LV LS and RV free wall LS were associated with the events [hazard ratio (HR) 0.72, P < 0.001 and HR: 0.83, P = 0.006, respectively]. Patients with impaired biventricular function had significantly shorter event-free survival than those with preserved biventricular function (P < 0.001).
CONCLUSION:
Deterioration of biventricular strain was associated with CS-related events. This information might be useful for clinical evaluation and follow-up in sarcoidosis.
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com.
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Pathological Overlap of Arrhythmogenic Right Ventricular Cardiomyopathy and Cardiac Sarcoidosis.
Circ Genom Precis Med2019 Oct;12(10):452-454. doi: 10.1161/CIRCGEN.119.002638.
Kerkar Ashwini, Hazard Florette, Caleshu Colleen, Shah Rajan, Reuter Chloe, Ashley Euan, Parikh Victoria N,
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Long-Term Corticosteroid-Sparing Immunosuppression for Cardiac Sarcoidosis.
J Am Heart Assoc2019 Sep;8(18):e010952. doi: 10.1161/JAHA.118.010952.
Rosenthal David G, Parwani Purvi, Murray Tyler O, Petek Bradley J, Benn Bryan S, De Marco Teresa, Gerstenfeld Edward P, Janmohamed Munir, Klein Liviu, Lee Byron K, Moss Joshua D, Scheinman Melvin M, Hsia Henry H, Selby Van, Koth Laura L, Pampaloni Miguel H, Zikherman Julie, Vedantham Vasanth,
Abstract
Background Long-term corticosteroid therapy is the standard of care for treatment of cardiac sarcoidosis (CS). The efficacy of long-term corticosteroid-sparing immunosuppression in CS is unknown. The goal of this study was to assess the efficacy of methotrexate with or without adalimumab for long-term disease suppression in CS, and to assess recurrence and adverse event rates after immunosuppression discontinuation. Methods and Results Retrospective chart review identified treatment-naive CS patients at a single academic medical center who received corticosteroid-sparing maintenance therapy. Demographics, cardiac uptake of 18-fluorodeoxyglucose, and adverse cardiac events were compared before and during treatment and between those with persistent or interrupted immunosuppression. Twenty-eight CS patients were followed for a mean 4.1 (SD 1.5) years. Twenty-five patients received 4 to 8 weeks of high-dose prednisone (>30 mg/day), followed by taper and maintenance therapy with methotrexate±low-dose prednisone (low-dose prednisone, <10 mg/day). Adalimumab was added in 19 patients with persistently active CS or in those with intolerance to methotrexate. Methotrexate±low-dose prednisone resulted in initial reduction (88%) or elimination (60%) of 18-fluorodeoxyglucose uptake, and patients receiving adalimumab-containing regimens experienced improved (84%) or resolved (63%) 18-fluorodeoxyglucose uptake. Radiologic relapse occurred in 8 of 9 patients after immunosuppression cessation, 4 patients on methotrexate-containing regimens, and in no patients on adalimumab-containing regimens. Conclusions Corticosteroid-sparing regimens containing methotrexate with or without adalimumab is an effective maintenance therapy in patients after an initial response is confirmed. Disease recurrence in patients on and off immunosuppression support need for ongoing radiologic surveillance regardless of immunosuppression regimen.
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Cardiac sarcoidosis in a patient with testicular seminoma.
BMJ Case Rep2019 Sep;12(9):. doi: e229912.
Bhatti Prashan, Waight Michael, Bromage Daniel, Sado Daniel,
Abstract
Sarcoidosis is a multisystem disorder characterised by non-caseating granulomas that typically affect the lungs, skin and lymph nodes. Sarcoidosis has been associated with various cancers, and we describe the case of a patient with systemic sarcoidosis associated with testicular seminoma. This was originally diagnosed as stable sarcoid-like reaction. He subsequently presented with ventricular tachycardia. Cardiovascular MRI suggested cardiac sarcoidosis, which was confirmed by myocardial biopsy. This case highlights the association between some types of cancer and sarcoidosis. In addition, it highlights the importance of close follow-up for patients with a history of malignancy to monitor for sarcoid-like reactions and sarcoidosis, which are often difficult to differentiate clinically.
© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.
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Inflammasome Formation in Granulomas in Cardiac Sarcoidosis.
Circ Arrhythm Electrophysiol2019 Sep;12(9):e007582. doi: 10.1161/CIRCEP.119.007582.
Kron Jordana, Mauro Adolfo Gabriele, Bonaventura Aldo, Toldo Stefano, Salloum Fadi N, Ellenbogen Kenneth A, Abbate Antonio,
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Localized basal left ventricular dyssynchrony induced by manifest accessory pathway: Successful differentiation from cardiac involvement of sarcoidosis with administration of flecainide acetate.
J Electrocardiol2019 Sep;57():87-89. doi: S0022-0736(19)30607-7.
Tsuda Daisuke, Mori Shumpei, Yokota Shun, Izawa Yu, Shimoyama Shinsuke, Suzuki Masataka, Takahashi Yu, Toh Hiroyuki, Toba Takayoshi, Tanaka Hidekazu, Fujiwara Sei, Hirata Ken-Ichi,
Abstract
We present a patient with non-cardiac sarcoidosis complicated with manifest ventricular preexcitation. Initially, cardiac involvement of sarcoidosis was suspected from the echocardiographic findings showing localized hypokinesia at the left ventricular basal inferior wall. We, however, considered that the hypokinesia was a preexcitation-induced mechanical dyssynchrony rather than cardiac sarcoidosis, because polarities of the delta-waves indicated a left ventricular inferior accessory pathway. Temporal administration of oral flecainide acetate eliminated the basal left ventricular motion abnormality. Accordingly, we could successfully differentiate the mechanism of hypokinesia. In this context, we could rule out cardiac sarcoidosis, and initiation of glucocorticoid therapy was reasonably withheld.
Copyright © 2019 Elsevier Inc. All rights reserved.
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