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Background: Sarcoidosis is a condition of unknown etiology. A number of occupational, recreational, and environmental exposures have been associated with the development of extra-cardiac sarcoidosis. Patients with clinically manifest cardiac sarcoidosis (CS) have a distinct clinical phenotype. We sought to explore the exposures associated with clinically manifest CS. Methods: Two groups of patients were recruited in a prospective registry: cases (patients with clinically manifest CS) and controls (patients without sarcoidosis and who had similar cardiac presentations to cases). A validated survey, previously used in other sarcoidosis phenotypes, was sent to all patients. Results: A total of 113 patients met the inclusion criteria and were sent the survey, of whom 79 of 113 (69.9%) completed the survey. We found 3 environmental associations. First, we found a negative association of CS with smoking, with 8 of 43 (18.6%) CS patients being current or ex-smokers compared to 17 of 36 (47.2%) of the controls. Second, we found a positive association with mold exposure, with 21 of 43 (48.8%) CS patients having a prior history of mold exposure compared to 9 of 36 (25.0%) of the controls. After multivariable analysis, there remained significant associations between CS and smoking (odds ratio 0.14 [95% confidence interval 0.04-0.51], P = 0.002) and mold exposure (odds ratio 5.69 [95% confidence interval 1.68-19.25], P = 0.005). Finally, patients with CS and self-reported acne had a significantly longer duration of active acne (7.82 ± 3.97 years) than did control patients 2.67 ± 1.03 years (P = 0.006). Conclusions: We found a negative association between smoking history and the diagnosis of CS. We also found a significant 5-fold increase in mold exposure and a positive association with duration of acne in patients with CS compared to controls. Résumé: Contexte: Les causes de la sarcoïdose demeurent inconnues. Un certain nombre de facteurs de risque professionnels, récréatifs et environnementaux ont toutefois été associés à la survenue de la sarcoïdose extracardiaque. Les patients qui sont atteints d’une sarcoïdose cardiaque cliniquement manifeste ont un phénotype clinique particulier. Nous nous sommes penchés sur les facteurs de risque associés à cette maladie. Méthodologie: Nous avons recruté deux groupes de patients à partir d’un registre de données prospectives : des cas de sarcoïdose cardiaque (présentant les manifestations cliniques de la sarcoïdose cardiaque) et des cas témoins (patients sans sarcoïdose, mais qui présentaient des signes et des symptômes cardiaques similaires à ceux des patients atteints de sarcoïdose cardiaque). Nous avons envoyé à l’ensemble des participants un questionnaire validé et déjà utilisé auprès de patients présentant d’autres phénotypes de sarcoïdose. Résultats: Au total, 113 patients répondaient aux critères d’inclusion. Nous avons envoyé le questionnaire à ces 113 patients, et 79 d’entre eux (69,9 %) y ont répondu. Nous avons détecté trois facteurs environnementaux. Nous avons d’abord noté une association négative entre la sarcoïdose cardiaque et le tabagisme : 8 patients atteints de sarcoïdose cardiaque sur 43 (18,6 %) fumaient ou avaient déjà fumé, comparativement à 17 témoins sur 36 (47,2 %). Nous avons ensuite observé une association positive avec l’exposition aux moisissures : 21 patients atteints de sarcoïdose cardiaque sur 43 (48,8 %) avaient des antécédents d’une telle exposition, comparativement à 9 des 36 témoins (25,0 %). Les associations entre la sarcoïdose cardiaque et le tabagisme et l’exposition aux moisissures sont demeurées significatives après une analyse multivariée (risque relatif approché [RRA] pour le tabagisme : 0,14 [intervalle de confiance {IC} à 95 % : 0,04-0,51], p = 0,002; RRA pour l’exposition aux moisissures : 5,69 [IC à 95 % : 1,68-19,25], p = 0,005). Finalement, chez les patients atteints de sarcoïdose cardiaque et d’acné autodéclarée, la durée de l’acné était significativement plus longue (7,82 ± 3,97) que celle relevée chez les témoins (2,67 ± 1,03; p = 0,006). Conclusions: Nous avons détecté une association négative entre les antécédents de tabagisme et le diagnostic de sarcoïdose cardiaque. Nous avons également observé que l’exposition aux moisissures était 5 fois plus élevée et que l’acné est présente plus longtemps chez les patients atteints de sarcoïdose cardiaque que chez les témoins.

INTRODUCTION We sought to explore the relationship between ventricular tachycardia (VT) and premature ventricular complex (PVC) burden (from implantable cardioverter-defibrillator diagnostics), before and during corticosteroid use in patients with newly diagnosed clinically manifest cardiac sarcoidosis (CS). METHODS A single-centre, prospective cohort study was performed in consecutive patients who met all of the following criteria: (1) presentation with clinically manifest CS, (2) abnormal myocardial fluoro-deoxyglucose (FDG) uptake on positron emission tomography scan, (3) plan for implantation with implantable cardioverter-defibrillator device that reports accurate PVC count, (4) plan to initiate corticosteroids after the device healing period. Data were collected during each device interrogation visit for all patients in the study. For each inter-visit period the total number of episodes of VT-sustained and nonsustained, and the number of PVCs was obtained. Each inter-visit period was classified into one of the following three periods: (1) New diagnosis of treatment-naive active disease without corticosteroids during the period. (2) Known treatment-naive active disease with corticosteroids initiated during the inter-visit period. (3) On corticosteroid therapy during the entire period. RESULTS A total of 20 patients with a mean age of 59.7 ± 7.7 years were recruited and 82 inter-visit periods were analyzed. All patients were corticosteroid responders based on FDG uptake. The maximum left ventricular standardized uptake value was 11.14 ± 5.19 before corticosteroid initiation and 4.07 ± 0.88 after (p

Cardiac sarcoidosis (CS) is an increasingly recognized condition, but cardiac magnetic resonance (CMR) image interpretation in these patients may be challenging as findings are often non-specific. The main objective of this study was to investigate the inter-reader agreement for the overall interpretation of CMR for the diagnosis of CS in an experienced reference center and investigate factors that may lead to discrepancies between readers. Consecutive patients undergoing CMR imaging to investigate for CS were included. CMR images were independently reviewed by two readers, blinded to all clinical, imaging and demographic information. The readers classified each scan as "consistent with cardiac sarcoidosis", "not consistent with cardiac sarcoidosis" or "indeterminate". Inter-reader agreement was assessed using κ-statistics. When there was disagreement on the overall interpretation, a third reader reviewed the images. Also, two readers independently commented on the presence of edema, presence of LGE (both ventricles) and quantified the extent of left ventricular LGE. 87 patients (43 women, mean age 54.3 ± 12.2 years) were included in the study. There was agreement regarding the overall interpretation in 72 of 87 (83%) CMR scans. The κ value was 0.64, indicating moderate agreement. There was similar moderate agreement in the interpretation of LGE parameters. In an experienced referral center, we found moderate agreement between readers in the interpretation of CMR in patients with suspected CS. Physicians should be aware of this inter-observer variability in interpretation of CMR studies in patients with suspected CS.

Background Recent data have suggested a substantial incidence of atrial arrhythmias (AAs) in cardiac sarcoidosis (CS). Our study aims were to first assess how often AAs are the presenting feature of previously undiagnosed CS. Second, we used prospective follow‐up data from implanted devices to investigate AA incidence, burden, predictors, and response to immunosuppression. Methods and Results This project is a substudy of the CHASM‐CS (Cardiac Sarcoidosis Multicenter Prospective Cohort Study; NCT01477359). Inclusion criteria were presentation with clinically manifest cardiac sarcoidosis, treatment‐naive status, and implanted with a device that reported accurate AA burden. Data were collected at each device interrogation visit for all patients and all potential episodes of AA were adjudicated. For each intervisit period, the total AA burden was obtained. A total of 33 patients met the inclusion criteria (aged 56.1±7.7 years, 45.5% women). Only 1 patient had important AAs as a part of the initial CS presentation. During a median follow‐up of 49.1 months, 11 of 33 patients (33.3%) had device‐detected AAs, and only 2 (6.1%) had a clinically significant AA burden. Both patients had reduced burden after CS was successfully treated and there was no residual fluorodeoxyglucose uptake on positron emission tomography scan. Conclusions First, we found that AAs are a rare presenting feature of clinically manifest cardiac sarcoidosis. Second, AAs occurred in a minority of patients at follow‐up; the burden was very low in most patients. Only 2 patients had clinically significant AA burden, and both had a reduction after CS was treated. Registration URL: https://www.clini​caltr​ials.gov ; unique identifier NCT01477359.

Aims There is ongoing controversy about the need for routine transoesophageal echocardiography (TOE) prior to atrial fibrillation (AF) ablation. Recently, the debate was reignited by the publication of a large series of patients showing a prevalence of left atrial appendage thrombus (LAAT) on TOE of 4.4%. We sought to assess the prevalence of LAAT on TOE before AF ablation at our institution. Methods and results Consecutive patients scheduled for AF ablation at our institution between January 2009 and December 2016 were included. All patients were on oral anticoagulation for at least 4 weeks prior to TOE. Transoesophageal echocardiographies were performed 3-5 days prior to scheduled AF ablation. Data were collected utilizing a prospective database. In all, 668 patients and 943 AF ablation procedures were included. Mean age was 64 ± 11 years, 72% were male, average CHADS2 score was 1.0 ± 1.0, and 72% of the patients had paroxysmal AF. At the time of ablation, 496 (53%) were on non-vitamin K antagonist oral anticoagulants (NOACs) and 447 (47%) were on Warfarin. There were three cases with LAAT (3/943, 0.3%), all of whom had persistent AF and were on Warfarin. Two patients underwent surgical ablation and the third patient did not undergo ablation. Conclusion In our experience, the prevalence of LAAT in patients on anticoagulation therapy undergoing TOE before catheter ablation of AF is 0.3%, which was much lower than recently reported. None of the patients with paroxysmal AF or on NOACs were found to have LAAT. Rather than routine use of TOE prior to AF ablation, a risk-based approach should be considered.

Background and objective Contact force (CF) sensing is a novel technology used for catheter ablation of atrial fibrillation (AF). We compared the single procedure success of CF-guided pulmonary vein isolation (PVI) with that of non-CF guided PVI during a 3-year (1,095 days) follow up period and analyzed the pattern of pulmonary vein (PV) reconnection. Methods A cohort of 167 subjects (68 CF vs. 99 non-CF) with paroxysmal AF were included in the study. Atrial arrhythmia (AA) recurrence was defined as documented AF, atrial flutter, or atrial tachycardia lasting >30 seconds and occurring after 90 days. Results Subjects in the CF group showed a statistically nonsignificant improvement in AA free survival compared to those in the non-CF group (66.2% vs. 51.5%; P value: 0.06). A greater propensity for reconnection was noted around the right-sided PVs compared to left-sided PVs related in both catheter ablation groups. For example, in the CF group 36% of right-sided segments reconnected compared to 16% of left-sided segments (P value

Presented is the case of a 62-year old male with a history of sarcoidosis and sinus node dysfunction, who underwent late gadolinium enhancement magnetic resonance imaging, which demonstrated left ventricular hyperenhancement and bi-atrial fibrosis.