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Pubblicazioni recenti - cardiac sarcoidosis
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Pulmonary rehabilitation for interstitial lung disease.
Cochrane Database Syst Rev2021 Feb;2():CD006322. doi: 10.1002/14651858.CD006322.pub4.
Dowman Leona, Hill Catherine J, May Anthony, Holland Anne E,
Abstract
BACKGROUND:
Interstitial lung disease (ILD) is characterised by reduced functional capacity, dyspnoea and exercise-induced hypoxia. Pulmonary rehabilitation is often used to improve symptoms, health-related quality of life and functional status in other chronic lung conditions. There is accumulating evidence for comparable effects of pulmonary rehabilitation in people with ILD. However, further information is needed to clarify the long-term benefit and to strengthen the rationale for pulmonary rehabilitation to be incorporated into standard clinical management of people with ILD. This review updates the results reported in 2014.
OBJECTIVES:
To determine whether pulmonary rehabilitation in people with ILD has beneficial effects on exercise capacity, symptoms, quality of life and survival compared with no pulmonary rehabilitation in people with ILD. To assess the safety of pulmonary rehabilitation in people with ILD.
SEARCH METHODS:
We searched CENTRAL, MEDLINE (Ovid), Embase (Ovid), CINAHL (EBSCO) and PEDro from inception to April 2020. We searched the reference lists of relevant studies, international clinical trial registries and respiratory conference abstracts to look for qualifying studies.
SELECTION CRITERIA:
We included randomised controlled trials and quasi-randomised controlled trials in which pulmonary rehabilitation was compared with no pulmonary rehabilitation or with other therapy in people with ILD of any origin.
DATA COLLECTION AND ANALYSIS:
Two review authors independently selected trials for inclusion, extracted data and assessed risk of bias. We contacted study authors to request missing data and information regarding adverse effects. We specified a priori subgroup analyses for participants with idiopathic pulmonary fibrosis (IPF) and participants with severe lung disease (low diffusing capacity or desaturation during exercise). There were insufficient data to perform the prespecified subgroup analysis for type of exercise training modality.
MAIN RESULTS:
For this update, we included an additional 12 studies resulting in a total of 21 studies. We included 16 studies in the meta-analysis (356 participants undertook pulmonary rehabilitation and 319 were control participants). The mean age of participants ranged from 36 to 72 years and included people with ILD of varying aetiology, sarcoidosis or IPF (with mean transfer factor of carbon dioxide (TLCO) % predicted ranging from 37% to 63%). Most pulmonary rehabilitation programmes were conducted in an outpatient setting, with a small number conducted in home-based, inpatient or tele-rehabilitation settings. The duration of pulmonary rehabilitation ranged from three to 48 weeks. There was a moderate risk of bias due to the absence of outcome assessor blinding and intention-to-treat analyses and the inadequate reporting of randomisation and allocation procedures in 60% of the studies. Pulmonary rehabilitation probably improves the six-minute walk distance (6MWD) with mean difference (MD) of 40.07 metres, 95% confidence interval (CI) 32.70 to 47.44; 585 participants; moderate-certainty evidence). There may be improvements in peak workload (MD 9.04 watts, 95% CI 6.07 to 12.0; 159 participants; low-certainty evidence), peak oxygen consumption (MD 1.28 mL/kg/minute, 95% CI 0.51 to 2.05; 94 participants; low-certainty evidence) and maximum ventilation (MD 7.21 L/minute, 95% CI 4.10 to 10.32; 94 participants; low-certainty evidence). In the subgroup of participants with IPF, there were comparable improvements in 6MWD (MD 37.25 metres, 95% CI 26.16 to 48.33; 278 participants; moderate-certainty evidence), peak workload (MD 9.94 watts, 95% CI 6.39 to 13.49; low-certainty evidence), VO (oxygen uptake) peak (MD 1.45 mL/kg/minute, 95% CI 0.51 to 2.40; low-certainty evidence) and maximum ventilation (MD 9.80 L/minute, 95% CI 6.06 to 13.53; 62 participants; low-certainty evidence). The effect of pulmonary rehabilitation on maximum heart rate was uncertain. Pulmonary rehabilitation may reduce dyspnoea in participants with ILD (standardised mean difference (SMD) -0.36, 95% CI -0.58 to -0.14; 348 participants; low-certainty evidence) and in the IPF subgroup (SMD -0.41, 95% CI -0.74 to -0.09; 155 participants; low-certainty evidence). Pulmonary rehabilitation probably improves health-related quality of life: there were improvements in all four domains of the Chronic Respiratory Disease Questionnaire (CRQ) and the St George's Respiratory Questionnaire (SGRQ) for participants with ILD and for the subgroup of people with IPF. The improvement in SGRQ Total score was -9.29 for participants with ILD (95% CI -11.06 to -7.52; 478 participants; moderate-certainty evidence) and -7.91 for participants with IPF (95% CI -10.55 to -5.26; 194 participants; moderate-certainty evidence). Five studies reported longer-term outcomes, with improvements in exercise capacity, dyspnoea and health-related quality of life still evident six to 12 months following the intervention period (6MWD: MD 32.43, 95% CI 15.58 to 49.28; 297 participants; moderate-certainty evidence; dyspnoea: MD -0.29, 95% CI -0.49 to -0.10; 335 participants; SGRQ Total score: MD -4.93, 95% CI -7.81 to -2.06; 240 participants; low-certainty evidence). In the subgroup of participants with IPF, there were improvements at six to 12 months following the intervention for dyspnoea and SGRQ Impact score. The effect of pulmonary rehabilitation on survival at long-term follow-up is uncertain. There were insufficient data to allow examination of the impact of disease severity or exercise training modality. Ten studies provided information on adverse events; however, there were no adverse events reported during rehabilitation. Four studies reported the death of one pulmonary rehabilitation participant; however, all four studies indicated this death was unrelated to the intervention received.
AUTHORS' CONCLUSIONS:
Pulmonary rehabilitation can be performed safely in people with ILD. Pulmonary rehabilitation probably improves functional exercise capacity, dyspnoea and quality of life in the short term, with benefits also probable in IPF. Improvements in functional exercise capacity, dyspnoea and quality of life were sustained longer term. Dyspnoea and quality of life may be sustained in people with IPF. The certainty of evidence was low to moderate, due to inadequate reporting of methods, the lack of outcome assessment blinding and heterogeneity in some results. Further well-designed randomised trials are needed to determine the optimal exercise prescription, and to investigate ways to promote longer-lasting improvements, particularly for people with IPF.
Copyright © 2021 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
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Is positron emission tomography enough to rule out cardiac sarcoidosis? A case report.
Eur Heart J Case Rep2021 Sep;5(9):ytab300. doi: 10.1093/ehjcr/ytab300.
Huang Siyi, Kunchakarra Siri, Rathod Ankit,
Abstract
Background:
Cardiac sarcoidosis (CS) is associated with poor prognosis, yet the clinical diagnosis is often challenging. Advanced cardiac imaging including cardiac magnetic resonance (CMR) and positron emission tomographic (PET) have emerged as useful modalities to diagnose CS.
Case summary:
A 66-year-old woman presented with palpitations. A 24-h Holter monitor detected a high premature ventricular contraction burden of 25.6%. She underwent two transthoracic echocardiograms; both showed normal results. Stress perfusion CMR did not show any evidence of ischaemic aetiology; however, myocardial lesions detected by late gadolinium enhancement (LGE) imaging raised suspicion for CS. While there was no myocardial uptake of fluorodeoxyglucose (FDG) in subsequent cardiac PET, high FDG uptake was seen in hilar lymph nodes. Lymph node biopsy confirmed the diagnosis of sarcoidosis.
Discussion:
Cardiac magnetic resonance and PET imaging are designed to evaluate different aspects CS pathophysiology. The characteristic LGE in the absence of increased FDG uptake suggested inactive CS with residual myocardial scarring.
© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Masquerading Clinical Features Associated With Sudden Cardiac Arrest in Sarcoidosis.
JACC Clin Electrophysiol -
Sex Differences in Patients With Suspected Cardiac Sarcoidosis Assessed by Cardiovascular Magnetic Resonance Imaging.
Circ Arrhythm Electrophysiol2021 Sep;14(9):e009966. doi: 10.1161/CIRCEP.121.009966.
Kalra Rajat, Malik Shray, Chen Ko-Hsuan Amy, Ogugua Fredrick, Athwal Pal Satyajit Singh, Elton Andrew C, Velangi Pratik S, Ismail Mohamed F, Chhikara Sanya, Markowitz Jeremy S, Nijjar Prabhjot S, von Wald Lisa, Roukoz Henri, Bhargava Maneesh, Perlman David, Shenoy Chetan,
Abstract
[Figure: see text].
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Abrupt Onset of Cardiac Tamponade in Sarcoidosis.
Int Heart J2021 Sep;():. doi: 10.1536/ihj.21-167.
Yano Toshiyuki, Hisahara Shin, Nagano Nobutaka, Noto Takahiro, Ogawa Toshifumi, Nishikawa Ryo, Koyama Masayuki, Kouzu Hidemichi, Muranaka Atsuko, Hashimoto Akiyoshi, Shimohama Shun, Miura Tetsuji,
Abstract
Sarcoidosis is a systemic inflammatory disease characterized by the formation of noncaseating epithelioid granulomas. Multiple organs, including the lung, eyes, and skin, are involved in this disorder, and cardiac involvement is a major cause of morbidity and mortality in patients with this disorder. We present the case history of a 22-year-old man with neurosarcoidosis complicated by abrupt onset of cardiac tamponade. Cardiac tamponade is a rare but potentially fatal manifestation of sarcoidosis, which is treatable with glucocorticoid therapy. Including the present case, previously reported cases of sarcoidosis with cardiac tamponade are reviewed to delineate its clinical characteristics.
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Cytokine Signaling and Matrix Remodeling Pathways Associated with Cardiac Sarcoidosis Disease Activity Defined Using FDG PET Imaging.
Int Heart J2021 Sep;():. doi: 10.1536/ihj.21-164.
Young Bryan D, Moreland Hannah, Oatmen Kelsie E, Freeburg Lisa A, Shahab Zartashia, Herzog Erica, Miller Edward J, Spinale Francis G,
Abstract
While cardiac imaging has improved the diagnosis and risk assessment for cardiac sarcoidosis (CS), treatment regimens have consisted of generalized heart failure therapies and non-specific anti-inflammatory regimens. The overall goal of this study was to perform high-sensitivity plasma profiling of specific inflammatory pathways in patients with sarcoidosis and with CS.Specific inflammatory/proteolytic cascades were upregulated in sarcoidosis patients, and certain profiles emerged for CS patients.Plasma samples were collected from patients with biopsy-confirmed sarcoidosis undergoing F-18 fluorodeoxyglucose positron emission tomography (n = 47) and compared to those of referent control subjects (n = 6). Using a high-sensitivity, automated multiplex array, cytokines, soluble cytokine receptor profiles (an index of cytokine activation), as well as matrix metalloproteinase (MMP), and endogenous MMP inhibitors (TIMPs) were examined.The plasma tumor necrosis factor (TNF) and soluble TNF receptors sCD30 and sTNFRI were increased using sarcoidosis, and sTNFRII increased in CS patients (n = 18). The soluble interleukin sIL-2R and vascular endothelial growth factor receptors (sVEGFR2 and sVEGFR3) increased to the greatest degree in CS patients. When computed as a function of referent control values, the majority of soluble cytokine receptors increased in both sarcoidosis and CS groups. Plasma MMP-9 levels increased in sarcoidosis but not in the CS subset. Plasma TIMP levels declined in both groups.The findings from this study were the identification of increased activation of a cluster of soluble cytokine receptors, which augment not only inflammatory cell maturation but also transmigration in patients with sarcoidosis and patients with cardiac involvement.
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Hypercalcaemic encephalopathy as a presenting manifestation of sarcoidosis.
BMJ Case Rep2021 Sep;14(9):. doi: e241246.
Ray Adrija, Kar Animesh, Ray Biman Kanti, Dubey Souvik,
Abstract
A 66-year-old woman presented to us with features of encephalopathy with asterixis, preceded by unsteadiness of gait and behavioural abnormalities. On subsequent investigations, hypercalcaemic crisis and compromised renal function were noted. Stepwise approach to determine the cause behind hypercalcaemia with compromised renal function revealed underlying granulomatous disease (sarcoidosis). Later, development of maculopapular rash and subsequent biopsy from the lesion confirmed the diagnosis of sarcoidosis. Her clinical and biochemical parameters improved considerably on initiation of conservative pharmacological therapy.
© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.
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A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc.
Best Pract Res Clin Rheumatol2021 Sep;():101707. doi: S1521-6942(21)00049-8.
Saketkoo Lesley Ann, Frech Tracy, Varjú Cecília, Domsic Robyn, Farrell Jessica, Gordon Jessica K, Mihai Carina, Sandorfi Nora, Shapiro Lee, Poole Janet, Volkmann Elizabeth R, Lammi Monika, McAnally Kendra, Alexanderson Helene, Pettersson Henrik, Hant Faye, Kuwana Masataka, Shah Ami A, Smith Vanessa, Hsu Vivien, Kowal-Bielecka Otylia, Assassi Shervin, Cutolo Maurizio, Kayser Cristiane, Shanmugam Victoria K, Vonk Madelon C, Fligelstone Kim, Baldwin Nancy, Connolly Kerri, Ronnow Anneliese, Toth Beata, Suave Maureen, Farrington Sue, Bernstein Elana J, Crofford Leslie J, Czirják László, Jensen Kelly, Hinchclif Monique, Hudson Marie, Lammi Matthew R, Mansour Jennifer, Morgan Nadia D, Mendoza Fabian, Nikpour Mandana, Pauling John, Riemekasten Gabriela, Russell Anne-Marie, Scholand Mary Beth, Seigart Elise, Rodriguez-Reyna Tatiana Sofia, Hummers Laura, Walker Ulrich, Steen Virginia,
Abstract
Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the extent possible, prevention of SSc and treatment-related complications.
Copyright © 2021 Elsevier Ltd. All rights reserved.
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Cardiac sarcoidosis: systematic review of literature on corticosteroid and immunosuppressive therapies.
Eur Respir J2021 Sep;():. doi: 2100449.
Stievenart Julien, Le Guenno Guillaume, Ruivard Marc, Rieu Virginie, André Marc, Grobost Vincent,
Abstract
BACKGROUND:
Cardiac sarcoidosis (CS) is a life-threatening condition in which clear recommendations are lacking. We aimed to review systematically the literature on cardiac sarcoidosis treated by corticosteroids and/or immunosuppressive agents in order to update the management of CS.
METHODS:
Using Pubmed, Embase and Cochrane Library databases, we found original articles on corticosteroid and/or standard immunosuppressive therapies for CS which provided at least fair SIGN overall assessment of quality and analyse the relapse rate, major cardiac adverse events (MACEs) and adverse events. We base our methods on Prisma statement and checklist.
RESULTS:
We retrieved 21 studies. Mean quality provided by SIGN assessment was 6.8/14 (range 5-9). Corticosteroids appeared to have a positive impact on left ventricular function, atrioventricular block, and ventricular arrhythmias. For corticosteroids alone, nine (45%) studies (n=351) provided data on relapses, representing an incidence of 34% (n=119). Three studies (14%, n=73) provided data on MACEs (n=33), representing 45% of MACEs in patients treated by corticosteroid alone. Nine studies provided data on adjunctive immunosuppressive therapy in which four studies (n=78) provided data on CS relapse, representing an incidence of 33% (n=26). Limitations consisted in no randomised control trial retrieved and unclear data on MACEs in patients treated by combined immunosuppressive agents and corticosteroids.
CONCLUSIONS:
Corticosteroids should be started early after diagnosis but the exact scheme is still unclear. Studies concerning adjunctive conventional immunosuppressive therapies are lacking and benefits of adjunctive immunosuppressive therapies are unclear. Homogenous data on CS long-term outcomes under corticosteroids, immunosuppressive therapies and other adjunctive therapies are lacking.
Copyright ©The authors 2021.
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Clinical Presentation and Treatment of High-Risk Sarcoidosis.
Ann Am Thorac Soc2021 Sep;():. doi: 10.1513/AnnalsATS.202102-212CME.
Perlman David M, Sudheendra Muthya Tejasvini, Furuya Yuka, Shenoy Chetan, Kalra Rajat, Roukoz Henri, Markowitz Jeremy, Maier Lisa A, Bhargava Maneesh,
Abstract
Sarcoidosis is a multisystem disease of unknown cause with heterogenous clinical manifestations and variable course. Spontaneous remissions occur in some patients while others have progressive disease impacting survival, organ function, and quality of life. Four high-risk sarcoidosis phenotypes associated with chronic inflammation have recently been identified as high-priority areas for research. These include treatment-refractory pulmonary disease, cardiac sarcoidosis, neurosarcoidosis and multiorgan sarcoidosis. Significant gaps currently exist in understanding of these high-risk manifestations of sarcoidosis, including their natural history, diagnostic criteria, biomarkers, and the treatment strategy such as the ideal agent, optimal dose and treatment duration. The use of registries with well-phenotyped patients is a critical first step to study high-risk sarcoidosis manifestations systematically. We review the diagnostic and treatment approach to high-risk sarcoidosis manifestations. Appropriately identifying these disease sub-groups will help enroll well-phenotyped patients in sarcoidosis registries and clinical trials, a necessary step to narrow existing gaps in understanding of this enigmatic disease.
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Association of high serum soluble interleukin 2 receptor levels with risk of adverse events in cardiac sarcoidosis.
ESC Heart Fail2021 Sep;():. doi: 10.1002/ehf2.13614.
Kobayashi Yuta, Sato Takuma, Nagai Toshiyuki, Hirata Kenji, Tsuneta Satonori, Kato Yoshiya, Komoriyama Hirokazu, Kamiya Kiwamu, Konishi Takao, Omote Kazunori, Ohira Hiroshi, Kudo Kohsuke, Konno Satoshi, Anzai Toshihisa,
Abstract
AIMS:
Although soluble interleukin 2 receptor (sIL-2R) is a potentially useful biomarker in the diagnosis and evaluation of disease severity in patients with sarcoidosis, its prognostic implication in patients with cardiac sarcoidosis (CS) is unclear. We sought to investigate whether sIL-2R was associated with clinical outcomes and to clarify the relationship between sIL-2R levels and disease activity in patients with CS.
METHODS AND RESULTS:
We examined 83 consecutive patients with CS in our hospital who had available serum sIL-2R data between May 2003 and February 2020. The primary outcome was a composite of advanced atrioventricular block, ventricular tachycardia or ventricular fibrillation, heart failure hospitalization, and all-cause death. Inflammatory activity in the myocardium and lymph nodes was assessed by F-fluorideoxyglucose positron emission tomography/computed tomography. During a median follow-up period of 2.96 (IQR 2.24-4.27) years, the primary outcome occurred in 24 patients (29%). Higher serum sIL-2R levels (>538 U/mL, the median) were significantly related to increased incidence of primary outcome (P = 0.037). Multivariable Cox regression analysis showed that a higher sIL-2R was independently associated with an increased subsequent risk of adverse events (HR 3.71, 95% CI 1.63-8.44, P = 0.002), even after adjustment for significant covariates. sIL-2R levels were significantly correlated to inflammatory activity in lymph nodes (r = 0.346, P = 0.003) but not the myocardium (r = 0.131, P = 0.27).
CONCLUSIONS:
Increased sIL-2R is associated with worse long-term clinical outcomes accompanied by increased systemic inflammatory activity in CS patients.
© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
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The role of multimodality imaging in diagnosing acute perimyocarditis secondary to Crohn's disease.
BMC Cardiovasc Disord2021 Sep;21(1):427. doi: 10.1186/s12872-021-02232-x.
Prameswari Hawani Sasmaya, Balakrishnan Iswaree Devi, Khoo Chun Yuan, Teo Loon Yee, Chan Lihua Laura, Ng Choon Ta,
Abstract
BACKGROUND:
Acute perimyocarditis is a rare extra-intestinal manifestation in Crohn's disease which required multimodality imaging to confirm the diagnosis. Here we present a case of acute perimyocarditis as the first presentation of Crohn's disease. To date, this is the first case presentation reporting the use of F-FDG PET/CT Scan for diagnosing such condition.
CASE PRESENTATION:
A 25-year-old male presented to our hospital with severe persistent pleuritic sharp left-sided chest pain. This was his second hospital admission in the past 4 months for chest pain and diarrhea. At the first hospitalization, he was diagnosed with viral perimyocarditis and irritable bowel syndrome. Laboratory findings, electrocardiogram, and cardiac magnetic resonance imaging results confirm the diagnostic of perimyocarditis. Virology, bacteriology, parasitology, and autoimmune evaluations were unremarkable. Colonoscopy, colorectal biopsy, and FGD PET findings confirmed manifestation of perimyocarditis, Crohn's disease, and negative for sarcoidosis.
CONCLUSIONS:
Looking at the overall clinical picture and investigation results of colonoscopy, colorectal biopsy findings, as well as multi-modality imaging with echocardiography, FDG PET-scan and CMRI, the patient was diagnosed to have perimyocarditis attending Chron's disease flare up as a rare extra-intestinal manifestation.
© 2021. The Author(s).
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The prognostic value of positron emission tomography in the evaluation of suspected cardiac sarcoidosis.
J Nucl Cardiol2021 Sep;():. doi: 10.1007/s12350-021-02780-x.
Patel Vaiibhav N, Pieper Justin A, Poitrasson-Rivière Alexis, Kopin David, Cascino Thomas, Aaronson Keith, Murthy Venkatesh L, Koelling Todd,
Abstract
OBJECTIVES:
To assess the prognostic value of positron emission tomography (PET) imaging in patients undergoing evaluation for known or suspected cardiac sarcoidosis (CS) while not on active immunotherapy.
BACKGROUND:
Previous studies have attempted to identify the value of PET imaging to aid in risk stratification of patients with CS, however, most cohorts have included patients currently on immunosuppression, which may confound scan results by suppressing positive findings.
METHODS:
We retrospectively analyzed 197 patients not on immunosuppression who underwent F-fluorodeoxyglucose (FDG) PET scans for evaluation of known or suspected CS. The primary endpoint of the study was time to ventricular arrhythmia (VT/VF), or death. Candidate predictors were identified by univariable Cox proportional hazards regression. Independent predictors were identified by performing multivariable Cox regression with stepwise forward selection.
RESULTS:
Median follow-up time was 531 [IQR 309, 748] days. 41 patients met the primary endpoint. After stepwise forward selection, left ventricular ejection fraction (LVEF) (HR 0.98, 95% CI 0.96-0.99, P = 0.02), history of VT/VF (HR 4.19, 95% CI 2.15-8.17, P
CONCLUSIONS:
Among untreated patients who underwent PET scans to evaluate known or suspected CS, LVEF, history of VT/VF, and SRS were associated with adverse clinical outcomes.
© 2021. American Society of Nuclear Cardiology.
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Case report of a peculiar aneurysm of the ascending aorta: when there is much more beyond an incidental finding.
Eur Heart J Case Rep2021 Aug;5(8):ytab205. doi: 10.1093/ehjcr/ytab205.
Ceschia Nicole, Scheggi Valentina, Marchionni Niccolò, Stefano Pierluigi,
Abstract
Background:
Aneurysms of the thoracic aorta are common in male patients around the VI-VII decade of life and most have a degenerative aetiology; otherwise, the occurrence of this disease at a younger age should prompt the search of rarer causes. We report a singular case of ascending aortic aneurysm (AAA) in a young man.
Case summary:
A large AAA accompanied by multivessel dilatation and renal failure of unknown onset was incidentally found in a 23-year-old male during the diagnostic work-up after a car accident. A systemic disease was therefore suspected, and a full clinical investigation revealed the uncommon diagnosis of sarcoidosis accompanied by large vessel vasculitis.
Discussion:
Only a few reports in the literature describe the concurrence of sarcoidosis and large vessel vasculitis (Takayasu arteritis), which may share non-specific immunoinflammatory abnormalities. This case underlines the importance of a multisystem diagnostic approach even in front of an incidental finding that is inconsistent with patient's age.
© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Characteristics and Outcomes of Patients with Inflammatory Cardiomyopathies Receiving Mechanical Circulatory Support: An STS-Intermacs Registry Analysis.
J Card Fail2021 Aug;():. doi: S1071-9164(21)00345-6.
Sheikh Farooq H, Craig Paige E, Ahmed Sara, Torguson Rebecca, Kolm Paul, Weintraub William S, Molina Ezequiel J, Najjar Samer S, Mohammed Selma F,
Abstract
BACKGROUND:
Durable mechanical circulatory support therapy (MCS) improves survival in patients with advanced heart failure. Knowledge regarding the outcomes experienced by inflammatory cardiomyopathy patients who receive durable MCS therapy is limited.
METHODS:
We compared patients with inflammatory cardiomyopathy to idiopathic dilated cardiomyopathy (IDCM) patients enrolled in the STS-Intermacs registry.
RESULTS:
Among 19,012 patients, 329 (1.7%) had inflammatory cardiomyopathy, whereas 5,978 had IDCM (31.4%). The inflammatory cardiomyopathy patients were younger, more likely to be white, and women. These patients experienced more pre-operative arrhythmias and higher use of temporary MCS. Inflammatory cardiomyopathy patients had a higher rate of early adverse events (
CONCLUSION:
Inflammatory cardiomyopathy patients who received durable MCS appear to have similar survival compared to idiopathic dilated cardiomyopathy patients despite a higher early adverse event burden. Our findings support the use of durable MCS in an inflammatory cardiomyopathy population.
Copyright © 2021. Published by Elsevier Inc.
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Corticosteroid and Immunosuppressant Therapy for Cardiac Sarcoidosis: A Systematic Review.
J Am Heart Assoc2021 Sep;10(17):e021183. doi: 10.1161/JAHA.121.021183.
Fazelpour Siavosh, Sadek Mouhannad M, Nery Pablo B, Beanlands Rob S, Tzemos Niko, Toma Mustafa, Birnie David H,
Abstract
Background Corticosteroid therapy for the treatment of clinically manifest cardiac sarcoidosis is generally recommended. Our group previously systematically reviewed the data in 2013; since then, there has been increasing quality and quantity of data and also interest in nonsteroid agents. Methods and Results Studies were identified from MEDLINE, EMBASE, Cochrane Controlled Trials Register, Cochrane Database of Systematic Reviews, and the National Institutes of Health ClinicalTrials.gov database. The quality of included articles was rated using Scottish Intercollegiate Guidelines Network 50. Outcomes examined were atrioventricular conduction, left ventricular function, ventricular arrhythmias, and mortality. A total of 3527 references were retrieved, and 34 publications met the inclusion criteria. There were no randomized trials, and only 2 studies were rated good quality. In the 34 reports (total of 1297 patients), 1125 patients received corticosteroids, 235 received additional or other immunosuppressant therapy, and 97 patients received no therapy. There were 178 patients treated for atrioventricular conduction disease, with 76/178 (42.7%) improving. In contrast, 21 patients were not treated with corticosteroids and/or immunosuppressant therapy, and none of them improved. Therapy was associated with the prevention of deterioration in left ventricular function. A total of 8 publications reported on ventricular arrhythmia burden, and 19 reported on mortality; the data quality was too limited to draw conclusions for the latter 2 outcomes. Conclusions The best quality data relate to atrioventricular nodal conduction and left ventricular function recovery. In both situations, therapy with corticosteroids and/or immunosuppressant therapy were sometimes associated with positive outcomes. The data quality is too limited to draw conclusions for ventricular arrhythmias and mortality.
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Clinical and Electrophysiological Characteristics of Ventricular Tachycardias From the Basal Septum in Structural Heart Disease.
JACC Clin Electrophysiol2021 Aug;():. doi: S2405-500X(21)00520-X.
Kotake Yasuhito, Campbell Timothy, Bennett Richard G, Turnbull Samual, Huang Kaimin, Ross Neil, Trivic Ivana, De Silva Kasun, Bhaskaran Ashwin, Kumar Saurabh,
Abstract
OBJECTIVE:
This study describes the clinical and electrophysiological characteristics of basal-septal ventricular tachycardias (VTs) in patients with structural heart disease (SHD).
BACKGROUND:
The basal septum is a common source of VT in patients with SHD.
METHODS:
Data from 312 consecutive patients with SHD undergoing catheter ablation of ventricular arrhythmias were reviewed.
RESULTS:
Thirty-three basal-septal VTs in 31 patients (mean age 67.4 ± 14.2 years, mean left ventricular ejection fraction [LVEF] 42% ± 15%) were identified. Patients with VTs with left ventricular basal-septal breakthrough were more likely to have ischemic cardiomyopathy and lower LVEF; patients with right ventricular basal-septal VT were more likely to have sarcoidosis or right ventricular cardiomyopathy of unknown significance, with higher LVEF. Atrioventricular block was present in 45% of patients and intraventricular block including persistent biventricular pacing in 77%. Unipolar scar was larger than bipolar scar (area 18.8% ± 19.4% vs 12.7% ± 14.6%; P
CONCLUSIONS:
Basal-septal VTs in patients with SHD have a distinct clinical, electrocardiographic, and electrophysiological profile depending on the breakthrough site, accompanied by a deep intramural septal substrate that limits procedural success after catheter ablation.
Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.
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A Polymorphism in C-C Chemokine Receptor 5 (CCR5) Associates with Löfgren's Syndrome and Alters Receptor Expression as well as Functional Response.
Cells2021 Aug;10(8):. doi: 1967.
Karakaya Bekir, van Moorsel Coline H M, Veltkamp Marcel, Roodenburg-Benschop Claudia, Kazemier Karin M, van der Helm-van Mil Annette H M, Huizinga Tom W J, Grutters Jan C, Rijkers Ger T,
Abstract
C-C chemokine receptor 5 (CCR5) and polymorphisms in gene are associated with sarcoidosis and Löfgren's syndrome. Löfgren's syndrome is an acute and usually self-remitting phenotype of sarcoidosis. We investigated whether the single nucleotide polymorphism (SNP) rs1799987 is associated with susceptibility for Löfgren's syndrome and has an effect on CCR5 expression on monocytes and function of CCR5. A total of 106 patients with Löfgren's syndrome and 257 controls were genotyped for rs1799987. Expression of CCR5 on monocytes was measured by flowcytometry. We evaluated calcium influx kinetics following stimulation upon N-formylmethionyl-leucyl-phenylalanine (fMLP) and macrophage inflammatory protein-1? (MIP-1?) on monocytes by measuring the median fluorescence intensity (MFI). The frequency of the G allele of rs1799987 was significantly higher in Löfgren's syndrome than in healthy controls ( = 0.0015, confidence interval (CI) 1.22-2.32, odds ratio (OR) 1.680). Patients with a GG genotype showed higher CCR5 expression on monocytes than patients with the AA genotype ( = 0.026). A significantly ( = 0.027) lower count of patients with the GG genotype showed a calcium influx reaction to simulation upon MIP-1 ?, compared with patients with the AA genotype. The rs1799987 G allele in gene is associated with susceptibility to Löfgren's syndrome and with quantitative and qualitative changes in CCR5, potentially effecting the inflammatory response.
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Myocardial findings evaluated by echocardiography in cardiac sarcoidosis: A report of seven cases.
J Clin Ultrasound2021 Aug;():. doi: 10.1002/jcu.23058.
Akamatsu Kanako, Ito Takahide, Terasaki Fumio, Hoshiga Masaaki,
Abstract
Sarcoidosis is a multisystem granulomatous disease of unknown cause. With cardiac sarcoidosis (CS), patients represent a wide range of cardiac manifestations from subtle to overt morphological and functional abnormalities. The advent of ultrasound technologies has enabled to identify not only typical findings to CS such as basal thinning of the ventricular septum, but also subclinical myocardial alterations. Based on our recent experiences, we currently introduce a variety of myocardial manifestations evaluated by echocardiography on seven CS patients being selected. Most of the patients exhibited typical cardiac involvement and the remaining fairly unusual.
© 2021 Wiley Periodicals LLC.
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Management of ventricular tachycardia in patients with cardiac sarcoidosis.
Heart Rhythm O22021 Aug;2(4):412-422. doi: 10.1016/j.hroo.2021.07.005.
Viwe Mtwesi, Nery Pablo, Birnie David H,
Abstract
Sarcoidosis is a multisystem granulomatous disease with 2 different phases (inflammation and scar). In the current era of targeted use of implantable cardioverter-defibrillators and modern heart failure therapy, recent data indicate the prognosis of cardiac sarcoidosis (CS) is much improved, and hence more patients are presenting with recurrent ventricular tachycardia (VT). This review highlights our current understanding of the pathophysiology and management of ventricular arrhythmias in CS with the major focus on indications, techniques, and outcomes of ablation. It is likely macroreentry phenomena around areas of fibrosis is the most frequent mechanism of ventricular arrhythmia in CS. It is also possible that inflammation may play a role in initiating reentry with ventricular ectopy in CS patients, or by slowing conduction in diseased tissue. The best available data would suggest annual rates of VT of perhaps 1%-2% and 10%-15% in patients with initially clinically silent and clinically manifest disease, respectively. Current guidelines recommend a stepwise approach to VT management. The first suggested step is treatment with immunosuppression if there is evidence of active inflammation. Antiarrhythmic medications are often started at the same time, with catheter ablation considered if VT cannot be controlled. Activation and entrainment mapping and ablation are favored in the setting of hemodynamically tolerated VT. Substrate ablation targets areas of abnormal electrogram and favorable pace mapping using linear and/or cluster lesion sets with the goal of abolishing critical isthmuses and/or blocking VT exit sites. Epicardial mapping ablation is required in 20%-35% of cases. In general, more morphologies of VT are induced (often 3-4) and subsequent outcomes (recurrence rates 40%-50%) are less favorable than in other forms of nonischemic cardiomyopathy. The prognosis of CS is much improved and, as a result, more patients are developing VT during follow-up. Likely principally related to the complex disease substrate, VT ablation is technically challenging, with moderate outcomes, and much remains to be learned.
© 2021 Heart Rhythm Society. Published by Elsevier Inc.
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