Arrhythmia Monitoring and Outcomes in Patients With Cardiac Sarcoidosis: Insights From the Cardiac Sarcoidosis Consortium.
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ABSTRACT:
SUBMITTER: Bressi E
PROVIDER: S-EPMC9333370 | biostudies-literature | 2022 Jul
REPOSITORIES: biostudies-literature
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Arrhythmia Monitoring and Outcomes in Patients With Cardiac Sarcoidosis: Insights From the Cardiac Sarcoidosis Consortium.
Bressi Edoardo E Crawford Thomas C TC Bogun Frank M FM Gu Xiaokui X Ellenbogen Kenneth A KA Chicos Alexandra B AB Roukoz Henri H Zimetbaum Peter J PJ Kalbfleisch Steven J SJ Murgatroyd Francis D FD Steckman David A DA Rosenfeld Lynda E LE Garlitski Ann C AC Soejima Kyoko K Bhan Adarsh K AK Vedantham Vasanth V Dickfeld Timm M TM De Lurgio David B DB Platonov Pyotr G PG Zipse Matthew M MM Nishiuchi Suguru S Ortman Matthew L ML Narasimhan Calambur C Patton Kris K KK Rosenthal David G DG Mukerji Siddharth S SS Hoogendoorn Jarieke C JC Zeppenfeld Katja K Sauer William H WH Kron Jordana J
Journal of the American Heart Association 20220622 13
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| S-EPMC9949704 | biostudies-literature
Project description:BackgroundVentricular arrhythmia (VA) is a life-threatening condition associated with cardiac sarcoidosis (CS). Right bundle branch block (RBBB) is a common conduction disorder in CS; however, its association with VA remains unknown.ObjectivesThis study aimed to investigate the relationship between RBBB and VA in patients with CS.MethodsThis was a post hoc analysis of ILLUMINATE-CS (Illustration of the Management and Prognosis of Japanese Patients with Cardiac Sarcoidosis), a multicenter, retrospective, and observational study that evaluated the clinical characteristics and prognosis of CS. Eligible patients were divided into two groups based on the presence or absence of RBBB at the time of diagnosis. The primary outcome was serious ventricular arrhythmia events (SVAEs), defined as a combination of sudden cardiac death and documented ventricular fibrillation, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator therapy.ResultsOverall, 312 patients were studied, with 155 (49.7%) patients presenting with RBBB (RBBB group). Patients in the RBBB group had a higher prevalence of basal interventricular septum (IVS) thinning and prominent late gadolinium enhancement in the basal IVS on cardiac magnetic resonance imaging than those in the non-RBBB group. During a median follow-up of 3.0 years (IQR: 1.6-6.0 years), 66 patients experienced SVAE. In multivariable Cox regression analysis, the RBBB group was independently associated with a higher incidence of SVAEs (HR: 1.93 [95% CI: 1.14-3.28]; P = 0.015).ConclusionsIn patients with CS, RBBB was an independent predictor of SVAEs, which might reflect the specific scar distribution that is predominant in the IVS.
| S-EPMC11299579 | biostudies-literature
Project description:Cardiac involvement in sarcoidosis has been reported to be as high as 50% of cases and it is well documented that it is associated with a poorer prognosis. Early recognition and treatment is key to reducing the risk of fatal arrhythmias and heart failure. We report a case of undiagnosed systemic sarcoidosis in a young man who initially presented to the emergency department with complete heart block in the context of preserved biventricular systolic function, and then again with ventricular tachycardia and moderately impaired left ventricular systolic function. We discuss how 18F-fluorodeoxyglucose positron emission tomography with CT was used for accurate diagnosis and how its role in the management of sarcoidosis has expanded with recent evidence suggesting it to be a mainstay of management and disease monitoring in cardiac sarcoidosis.
| S-EPMC4550861 | biostudies-other
Project description:ImportanceVentricular tachycardia (VT) is associated with high mortality in patients with cardiac sarcoidosis (CS), and medical management of CS-associated VT is limited by high failure rates. The role of catheter ablation has been investigated in small, single-center studies.ObjectiveTo investigate outcomes associated with VT ablation in patients with CS.Design, setting, and participantsThis cohort study from the Cardiac Sarcoidosis Consortium registry (2003-2019) included 16 tertiary referral centers in the US, Europe, and Asia. A total of 158 consecutive patients with CS and VT were included (33% female; mean [SD] age, 52 [11] years; 53% with ejection fraction [EF] <50%).ExposuresCatheter ablation of CS-associated VT and, as appropriate, medical treatment.Main outcomes and measuresImmediate and short-term outcomes included procedural success, elimination of VT storm, and reduction in defibrillator shocks. The primary long-term outcome was the composite of VT recurrence, heart transplant (HT), or death.ResultsComplete procedural success (no inducible VT postablation) was achieved in 85 patients (54%). Sixty-five patients (41%) had preablation VT storm that did not recur postablation in 53 (82%). Defibrillator shocks were significantly reduced from a median (IQR) of 2 (1-5) to 0 (0-0) in the 30 days before and after ablation (P < .001). During median (IQR) follow-up of 2.5 (1.1-4.9) years, 73 patients (46%) experienced VT recurrence and 81 (51%) experienced the composite primary outcome. One- and 2-year rates of survival free of VT recurrence, HT, or death were 60% and 52%, respectively. EF less than 50% and myocardial inflammation on preprocedural 18F-fluorodeoxyglucose positron emission tomography were significantly associated with adverse prognosis in multivariable analysis for the primary outcome (HR, 2.24; 95% CI, 1.37-3.64; P = .001 and HR, 2.93; 95% CI, 1.31-6.55; P = .009, respectively). History of hypertension was associated with a favorable long-term outcome (adjusted HR, 0.51; 95% CI, 0.28-0.92; P = .02).Conclusions and relevanceIn this observational study of selected patients with CS and VT, catheter ablation was associated with reductions in defibrillator shocks and recurrent VT storm. Preablation LV dysfunction and myocardial inflammation were associated with adverse long-term prognosis. These data support the role of catheter ablation in conjunction with medical therapy in the management of CS-associated VT.
| S-EPMC8600457 | biostudies-literature
Project description:Background: Definitions of cardiac sarcoidosis (CS) differ among guidelines. Any systemic histological finding of CS is essential for the diagnosis of CS in the 2014 Heart Rhythm Society statement, but not necessary in the Japanese Circulation Society 2016 guidelines. This study aimed to reveal the differences in outcomes by comparing 2 groups, namely CS patients with or without systemic histologically proven granuloma. Methods and Results: This study retrospectively included 231 consecutive patients with CS. CS with granulomas in ≥1 organs was diagnosed in 131 patients (Group G), whereas CS without any granulomas was diagnosed in the remaining 100 patients (Group NG). Left ventricular ejection fraction (LVEF) was significantly reduced in Group NG compared with Group G (44±13% vs. 50±16%, respectively; P=0.001). However, Kaplan-Meier curves showed that major adverse cardiovascular events (MACE)-free survival outcomes were comparable between the 2 groups (log-rank P=0.167). Univariable analyses showed that significant predictors of MACE were Groups G/NG, histological CS, LVEF, and high B-type natriuretic peptide (BNP) or N-terminal pro BNP concentrations, but none of these was significant in multivariable analyses. Conclusions: Overall risks of MACE were similar between the 2 groups despite different manifestations in cardiac dysfunction. The data not only validate the prognostic value of non-invasive diagnosis of CS, but also show the need for careful observation and therapeutic strategy in patients with CS without any granuloma.
| S-EPMC10247353 | biostudies-literature
Project description:ImportanceIn patients with sarcoidosis with suspected cardiac involvement, late gadolinium enhancement (LGE) on cardiovascular magnetic resonance imaging (CMR) identifies those with an increased risk of adverse outcomes. However, these outcomes are experienced by only a minority of patients with LGE, and identifying this subgroup may improve treatment and outcomes in these patients.ObjectiveTo assess whether CMR phenotypes based on left ventricular ejection fraction (LVEF) and LGE in patients with suspected cardiac sarcoidosis (CS) are associated with adverse outcomes during follow-up.Design, setting, and participantsThis cohort study included consecutive patients with histologically proven sarcoidosis who underwent CMR for the evaluation of suspected CS from 2004 to 2020 with a median follow-up of 4.3 years at an academic medical center in Minnesota. Demographic data, medical history, comorbidities, medications, and outcome data were collected blinded to CMR data.ExposuresCMR phenotypes were identified based on LVEF and LGE presence and features. LGE was classified as pathology-frequent or pathology-rare based on the frequency of cardiac damage features on gross pathology assessment of the hearts of patients with CS who had sudden cardiac death or cardiac transplant.Main outcomes and measuresComposite of ventricular arrhythmic events and composite of heart failure events.ResultsAmong 504 patients (mean [SD] age, 54.1 [12.5] years; 242 [48.0%] female and 262 [52.0%] male; 2 [0.4%] American Indian or Alaska Native, 6 [1.2%] Asian, 90 [17.9%] Black or African American, 399 [79.2%] White, 5 [1.0%] of 2 or more races (including the above-mentioned categories and Native Hawaiian or Other Pacific Islander), and 2 [0.4%] of unknown race; 4 [0.8%] Hispanic or Latino, 498 [98.8%] not Hispanic or Latino, and 2 [0.4%] of unknown ethnicity), 4 distinct CMR phenotypes were identified: normal LVEF and no LGE (n = 290; 57.5%), abnormal LVEF and no LGE (n = 53; 10.5%), pathology-frequent LGE (n = 103; 20.4%), and pathology-rare LGE (n = 58; 11.5%). The phenotype with pathology-frequent LGE was associated with a high risk of arrhythmic events (hazard ratio [HR], 12.12; 95% CI, 3.62-40.57; P < .001) independent of LVEF and extent of left ventricular late gadolinium enhancement (LVLGE). It was also associated with a high risk of heart failure events (HR, 2.49; 95% CI, 1.19-5.22; P = .02) independent of age, pulmonary hypertension, LVEF, right ventricular ejection fraction, and LVLGE extent. Risk of arrhythmic events was greater with an increasing number of pathology-frequent LGE features. The absence of the pathology-frequent LGE phenotype was associated with a low risk of arrhythmic events, even in the presence of LGE or abnormal LVEF.Conclusions and relevanceThis cohort study found that a CMR phenotype involving pathology-frequent LGE features was associated with a high risk of arrhythmic and heart failure events in patients with sarcoidosis. The findings indicate that CMR phenotypes could be used to optimize clinical decision-making for treatment options, such as implantable cardioverter-defibrillators.
| S-EPMC9475438 | biostudies-literature
Project description:PURPOSE OF REVIEW:In this state-of-the-art review, we highlight our current understanding of diagnosis, assessment, and management of cardiac sarcoidosis (CS), focusing on recently published data and expert consensus statement guidelines. RECENT FINDINGS:Academic interest in cardiac sarcoidosis research has increased over the past decade along with increased clinical awareness among clinicians. In 2014, the Heart Rhythm Society published the first expert consensus statement on diagnosing and managing arrhythmias associated with CS. Cardiac magnetic resonance has emerged as a valuable tool both for diagnosing CS and predicting risk of life-threatening ventricular arrhythmias based on burden of late gadolinium enhancement. Cardiac fluorodeoxyglucose-positron emission tomography now plays a role in diagnosis, risk stratification, and assessing response to immunosuppressive therapy. Collaborative, multidisciplinary research efforts are needed to further our understanding of this rare, complex disease. Two large multicenter prospective registries-the international Cardiac Sarcoidosis Consortium and the Canadian Cardiac Sarcoidosis Research Group-are enrolling patients to help provide insights into the natural history of the disease and current treatment strategies. Future research should focus on randomized controlled trials comparing different treatment strategies and identifying and testing novel therapeutic agents.
| S-EPMC7092931 | biostudies-literature
Project description:History of syncope is an independent predictor for sudden cardiac death. Programmed stimulation may be considered for risk stratification, but data remain sparse among different populations. Here, we analyzed the prognostic value of inducible ventricular arrhythmia (VA) regarding clinical outcome in patients with syncope undergoing defibrillator implantation. Among 4196 patients enrolled in the prospective, multi-center German Device Registry, patients with syncope and inducible VA (n = 285, 6.8%) vs. those with a secondary preventive indication (n = 1885, 45.2%), defined as previously documented sustained ventricular tachycardia or ventricular fibrillation, serving as a control group were studied regarding demographics, device implantation and post-procedural adverse events. Patients with syncope and inducible VA (64.9 ± 14.4 years, 81.1% male) presented less frequently with congestive heart failure (15.1% vs. 29.1%; p < 0.001) and any structural heart disease (84.9% vs. 89.3%; p = 0.030) than patients with a secondary preventive indication (65.0 ± 13.8 years, 81.0% male). Whereas dilated cardiomyopathy (16.8% vs. 23.8%; p = 0.009) was less common, hypertrophic cardiomyopathy (5.6% vs. 2.8%; p = 0.010) and Brugada syndrome (2.1% vs. 0.3%; p < 0.001) were present more often. During 1-year-follow-up, mortality (5.1% vs. 8.9%; p = 0.036) and the rate of major adverse cardiac or cerebrovascular events (5.8% vs. 10.0%; p = 0.027) were lower in patients with syncope and inducible VA. Among patients with inducible VA, post-procedural adverse events including rehospitalization (27.6% vs. 21.7%; p = 0.37) did not differ between those with vs. without syncope. Taken together, patients with syncope and inducible VA have better clinical outcomes than patients with a secondary preventive defibrillator indication, but comparable outcomes to patients without syncope, which underlines the relevance of VA inducibility, potentially irrespective of a syncope.
| S-EPMC10374635 | biostudies-literature
Project description:AimsThis study aimed to investigate the relationship between corticosteroid therapy and long-term outcomes in patients with cardiac sarcoidosis, stratified by left ventricular ejection fraction (LVEF) at diagnosis.Methods and resultsThis study conducted a post hoc analysis of the ILLUstration of the Management and prognosIs of JapaNese PATiEnts with Cardiac Sarcoidosis, a retrospective multicentre registry. Cardiac sarcoidosis was diagnosed based on the 2016 Japanese Circulation Society and 2014 Heart Rhythm Society criteria. The primary endpoint was a composite of all-cause death, hospitalization for heart failure, and fatal ventricular arrhythmia events. Patients were divided into three groups based on LVEF: preserved LVEF (≥50%, n = 251), moderately impaired LVEF (LVEF, 35-49%; n = 149), and severely impaired LVEF (<35%, n = 99). Among 499 patients with cardiac sarcoidosis (mean age: 61.6 ± 11.4 years, male: 36.1%), 419 (84.0%) were treated with corticosteroids after diagnosis. During a median follow-up of 33.7 months (interquartile range, 16.8-62.7 months), 144 primary endpoints (28.9%) occurred. Corticosteroid therapy was associated with better prognosis when assessed in terms of primary endpoint in the entire cohort [hazard ratio (HR) 0.61, 95% confidence interval (CI) 0.41-0.89, P = 0.010]. When stratified by LVEF, corticosteroid therapy was significantly associated with a lower incidence of primary endpoints in the preserved LVEF group (HR, 0.30; 95% CI, 0.15-0.57, P < 0.001), but not in the moderately and severely impaired LVEF groups. This association remained robust, even after adjusting for confounders.ConclusionIn this large cohort of cardiac sarcoidosis, corticosteroid therapy was associated with a lower incidence of long-term outcomes only in patients with preserved LVEF at diagnosis.Clinical trial registrationUMIN000034974.
| S-EPMC11720171 | biostudies-literature