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Brazile TL, Saul M, Nouraie SM, Gibson K. Characteristics and survival of patients diagnosed with cardiac sarcoidosis: A case series. Front Med (Lausanne) 2022; 9:1051412. [PMID: 36582282 PMCID: PMC9792839 DOI: 10.3389/fmed.2022.1051412] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2022] [Accepted: 11/22/2022] [Indexed: 12/15/2022] Open
Abstract
Background Sarcoidosis is a multiorgan system granulomatous disease of unknown etiology. It is hypothesized that a combination of environmental, occupational, and/or infectious factors provoke an immunological response in genetically susceptible individuals, resulting in a diversity of manifestations throughout the body. In the United States, cardiac sarcoidosis (CS) is diagnosed in 5% of patients with systemic sarcoidosis, however, autopsy results suggest that cardiac involvement may be present in > 50% of patients. CS is debilitating and significantly decreases quality of life and survival. Currently, there are no gold-standard clinical diagnostic or monitoring criteria for CS. Methods We identified patients with a diagnosis of sarcoidosis who were seen at the Simmons Center from 2007 to 2020 who had a positive finding of CS documented with cardiovascular magnetic resonance (CMR) and/or endomyocardial biopsy as found in the electronic health record. Medical records were independently reviewed for interpretation and diagnostic features of CS including late gadolinium enhancement (LGE) patterns, increased signal on T2-weighted imaging, and non-caseating granulomas, respectively. Extracardiac organ involvement, cardiac manifestations, comorbid conditions, treatment history, and vital status were also abstracted. Results We identified 44 unique patients with evidence of CS out of 246 CMR reports and 9 endomyocardial biopsy pathology reports. The first eligible case was diagnosed in 2007. The majority of patients (73%) had pulmonary manifestations, followed by hepatic manifestations (23%), cutaneous involvement (23%), and urolithiasis (20%). Heart failure was the most common cardiac manifestation affecting 59% of patients. Of these, 39% had a documented left ventricular ejection fraction of < 50% on CMR. Fifty eight percent of patients had a conduction disease and 44% of patients had documented ventricular arrhythmias. Pharmacotherapy was usually initiated for extracardiac manifestations and 93% of patients had been prescribed prednisone. ICD implantation occurred in 43% of patients. Patients were followed up for a median of 5.4 (IQR: 2.4-8.5) years. The 10-year survival was 70%. In addition to age, cutaneous involvement was associated with an increased risk of death (age-adjusted OR 8.47, 95% CI = 1.11-64.73). Conclusion CMR is an important tool in the non-invasive diagnosis of CS. The presence of LGE on CMR in a pattern consistent with CS has been shown to be a predictor of mortality and likely contributed to a high proportion of patients undergoing ICD implantation to decrease risk of sudden cardiac death. Clinical implications Additional studies are necessary to develop robust criteria for the diagnosis of CS with CMR, assess the benefit of serial imaging for disease monitoring, and evaluate the effect of immunosuppression on disease progression.
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Affiliation(s)
- Tiffany L. Brazile
- University of Pittsburgh School of Medicine, Pittsburgh, PA, United States
- University of Texas Southwestern Medical Center, Dallas, TX, United States
| | - Melissa Saul
- University of Pittsburgh School of Medicine, Pittsburgh, PA, United States
| | - Seyed Mehdi Nouraie
- University of Pittsburgh and The Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, Pittsburgh, PA, United States
| | - Kevin Gibson
- University of Pittsburgh and The Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, Pittsburgh, PA, United States
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2
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Hussain A, C Yiu A, A Okonkwo U, O'shea JP. Atrial fibrillation as a presenting symptom of Cardiac Sarcoid. J Atr Fibrillation 2021; 14:20200484. [PMID: 34950363 DOI: 10.4022/jafib.20200484] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Revised: 04/29/2021] [Accepted: 06/21/2021] [Indexed: 11/10/2022]
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 < 45 years-of-age, this case report aims to underscore consideration of cardiac sarcoidosis as a subclinical contributor towards developing atrial fibrillation in the appropriate patient population. Broadly more investigations are needed to explore the role of cardiac sarcoidosis with atrial involvement and the likelihood of developing atrial arrhythmias.
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Affiliation(s)
- Ali Hussain
- Department of Internal medicine, Tripler Army Medical Center, 1 Jarrett White Rd, Medical Center, HI 96859-5001, USA
| | - Alvin C Yiu
- Department of Internal medicine, Tripler Army Medical Center, 1 Jarrett White Rd, Medical Center, HI 96859-5001, USA
| | - Uzoagu A Okonkwo
- Department of Internal medicine, Tripler Army Medical Center, 1 Jarrett White Rd, Medical Center, HI 96859-5001, USA
| | - John-Paul O'shea
- Department of Internal medicine, Tripler Army Medical Center, 1 Jarrett White Rd, Medical Center, HI 96859-5001, USA
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3
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Tuominen H, Haarala A, Tikkakoski A, Kähönen M, Nikus K, Sipilä K. FDG-PET in possible cardiac sarcoidosis: Right ventricular uptake and high total cardiac metabolic activity predict cardiovascular events. J Nucl Cardiol 2021; 28:199-205. [PMID: 30815833 PMCID: PMC7920884 DOI: 10.1007/s12350-019-01659-2] [Citation(s) in RCA: 28] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2018] [Accepted: 02/01/2019] [Indexed: 12/14/2022]
Abstract
BACKGROUND Cardiac involvement accounts for the majority of morbidity and mortality in sarcoidosis. Pathological myocardial fluorodeoxyglucose (FDG)-uptake in positron emission tomography (PET) has been associated with cardiovascular events and quantitative metabolic parameters have been shown to add prognostic value. Our aim was to study whether the pattern of pathological cardiac FDG-uptake and quantitative parameters are able to predict cardiovascular events in patients with suspected cardiac sarcoidosis (CS). METHODS 137 FDG-PET examinations performed in Tampere University Hospital were retrospectively analyzed visually and quantitatively. Location of pathological uptake was noted and pathological metabolic volume, average standardized uptake value (SUV), and total cardiac metabolic activity (tCMA) were calculated. Patients were followed for ventricular tachycardia, decrease in left ventricular ejection fraction, and death. RESULTS Eleven patients had one or more cardiovascular events during the follow-up. Five patients out of 12 with uptake in both ventricles had an event during follow-up. Eight patients had high tCMA (> 900 MBq) and three of them had a cardiovascular event. Right ventricular uptake and tCMA were significantly associated with cardiovascular events during follow-up (P-value .001 and .018, respectively). CONCLUSIONS High tCMA and right ventricular uptake were significant risk markers for cardiac events among patient with suspected CS.
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Affiliation(s)
- Heikki Tuominen
- Department of Clinical Physiology and Nuclear Medicine, Tampere University Hospital, 33520, Tampere, Finland.
| | - Atte Haarala
- Department of Clinical Physiology and Nuclear Medicine, Tampere University Hospital, 33520, Tampere, Finland
| | - Antti Tikkakoski
- Department of Clinical Physiology and Nuclear Medicine, Tampere University Hospital, 33520, Tampere, Finland
| | - Mika Kähönen
- Department of Clinical Physiology and Nuclear Medicine, Tampere University Hospital, 33520, Tampere, Finland
- Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland
| | - Kjell Nikus
- Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland
- Department of Cardiology, Heart Center, Tampere University Hospital, Tampere, Finland
| | - Kalle Sipilä
- Department of Clinical Physiology and Nuclear Medicine, Tampere University Hospital, 33520, Tampere, Finland
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4
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Etinger R, Comaneshter D, Amital H, Cohen AD, Tiosano S. The long-term prognostic significance of heart failure in sarcoidosis patients ─ a cohort study. Postgrad Med 2020; 133:202-208. [PMID: 33019840 DOI: 10.1080/00325481.2020.1832773] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
OBJECTIVE To evaluate the proportion and the long-term prognostic significance of heart failure (HF) in sarcoidosis patients. METHODS Data extracted from a large Israeli healthcare provider's database were used to study sarcoidosis patients and matched non-sarcoidosis controls since 2000 to 2016. The proportion of HF was compared between the groups, and the associations between sarcoidosis, HF, and all-cause mortality were assessed. RESULTS Included were 3,993 sarcoidosis patients and 19,856 age- and sex-matched controls. The proportion of HF patients was higher among the former (10.9% and 5.3%, respectively). A logistic regression model for multivariable analysis for covariates found sarcoidosis to be independently associated with HF (Odds Ratio (OR) 2.09 confidence interval (CI) 1.83-2.39). A total of 710 sarcoidosis patients (17.8%) and 2,121 controls (10.7%) died during the study period (p < 0.001). A multivariable survival analysis found an estimated hazard ratio (HR) of 1.84 (95%CI 1.67-2.02), indicating a significant association between sarcoidosis and risk for all-cause mortality. Our analysis also revealed a significant association between HF and risk for all-cause mortality (HR 3.05, 95%CI 2.77-3.36). CONCLUSIONS Sarcoidosis is independently associated with HF, and both are independently associated with all-cause mortality.
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Affiliation(s)
- Roie Etinger
- Faculty of Medicine, Tel Aviv University Sackler, Tel Aviv, Israel
| | - Doron Comaneshter
- Department of Quality Measurements and Research, Clalit Health Services, Tel-Aviv, Israel
| | - Howard Amital
- Faculty of Medicine, Tel Aviv University Sackler, Tel Aviv, Israel.,Department of Internal Medicine B and Research Center for Autoimmune Diseases, The Chaim Sheba Medical Center at Tel Ha Shomer, Ramat-Gan, Israel
| | - Arnon D Cohen
- Department of Quality Measurements and Research, Clalit Health Services, Tel-Aviv, Israel.,Siaal Research Center for Family Medicine and Primary Care, Ben-Gurion University of the Negev Faculty of Health Sciences, Beer Sheva, Southern Israel
| | - Shmuel Tiosano
- Faculty of Medicine, Tel Aviv University Sackler, Tel Aviv, Israel.,The Leviev Heart Center, Sheba Medical Center, Tel-Hashomer, Israel
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5
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Association between sarcoidosis and cardiovascular comorbidity: A systematic review and meta-analysis. Heart Lung 2020; 49:512-517. [DOI: 10.1016/j.hrtlng.2020.03.013] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2019] [Revised: 03/09/2020] [Accepted: 03/12/2020] [Indexed: 11/17/2022]
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6
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Chazal T, Varnous S, Guihaire J, Goeminne C, Launay D, Boignard A, Vermes E, Dorent R, Camilleri L, Lelong B, Epailly E, Lebreton G, Waintraub X, Cluzel P, Maksud P, Fouret P, Leprince P, Grenier P, Amoura Z, Cohen Aubart F. Sarcoidosis diagnosed on granulomas in the explanted heart after transplantation: Results of a French nationwide study. Int J Cardiol 2020; 307:94-100. [DOI: 10.1016/j.ijcard.2019.12.066] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2018] [Revised: 12/01/2019] [Accepted: 12/30/2019] [Indexed: 01/13/2023]
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7
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Liauw D, Dang A, Littmann L. Wide QRS Complex Tachycardia in an Apparently Healthy Man. JAMA Intern Med 2020; 180:450-451. [PMID: 31930348 DOI: 10.1001/jamainternmed.2019.6694] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/14/2022]
Affiliation(s)
- Daniel Liauw
- University of North Carolina School of Medicine, Chapel Hill
| | - Anthony Dang
- Department of Internal Medicine, Atrium Health Carolinas Medical Center, Charlotte, North Carolina
| | - Laszlo Littmann
- Department of Internal Medicine, Atrium Health Carolinas Medical Center, Charlotte, North Carolina
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8
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Willy K, Dechering DG, Wasmer K, Köbe J, Bögeholz N, Ellermann C, Leitz P, Reinke F, Frommeyer G, Eckardt L. Outcome of catheter ablation of supraventricular tachyarrhythmias in cardiac sarcoidosis. Clin Cardiol 2019; 42:1121-1125. [PMID: 31482624 PMCID: PMC6837022 DOI: 10.1002/clc.23263] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/24/2019] [Accepted: 08/28/2019] [Indexed: 11/10/2022] Open
Abstract
Background Sarcoidosis is a multisystem granulomatous disease of not sufficiently understood origin. Some patients develop cardiac involvement in course of the disease which is mostly responsible for adverse outcome. In addition to complications like high degree atrioventricular (AV) block or ventricular tachyarrhythmias, there is a certain percentage of patients developing atrial tachyarrhythmias. Data is limited and the role of catheter ablation uncertain. Therefore, we studied sarcoid patients who presented with supraventricular tachyarrhythmias. Hypothesis Treatment and ablation of supraventricular tachycardia could be hampered by inflammation in patients with cardiac sarcoidosis. Methods We enrolled 37 consecutive patients with cardiac sarcoidosis who presented with atrial tachyarrhythmias and underwent an electrophysiologic study over a period of 6 years (03/2013‐04/2019). In total, 16 catheter ablations for atrial tachyarrhythmias were performed. Mean follow‐up duration was 2.5 years. Results Most common ablation performed was cavo‐tricuspid isthmus ablation for typical atrial flutter in seven patients (54%). Pulmonary vein isolation for treatment of atrial fibrillation (AF) was performed in five patients (38%). Two patients received slow‐pathway modulation for treatment of recurrent atrioventricular nodal reentry tachycardia (AVNRT). All but two patients with AF had no clinical recurrence during follow‐up. Two patients had recurrence of AF but still reported markedly improved european heart rhythm association (EHRA) class. Periprocedural safety was very high. There were no adverse events related to the ablation procedure. One patient died during follow‐up in the presence of electrical storm. Conclusion Catheter ablations of supraventricular tachycardias seem to be safe and effective in patients with cardiac sarcoidosis. Outcome is comparable to patients without inflammatory heart disease, although data from larger patient collectives are mandatory to make recommendations in this special entity.
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Affiliation(s)
- Kevin Willy
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | | | - Kristina Wasmer
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | - Julia Köbe
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | - Nils Bögeholz
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | - Christian Ellermann
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | - Patrick Leitz
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | - Florian Reinke
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | - Gerrit Frommeyer
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
| | - Lars Eckardt
- Department for Cardiology II: Electrophysiology, University Hospital Münster, Münster, Germany
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9
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Margaritopoulos GA, Kokosi MA, Wells AU. Diagnosing complications and co-morbidities of fibrotic interstitial lung disease. Expert Rev Respir Med 2019; 13:645-658. [PMID: 31215263 DOI: 10.1080/17476348.2019.1632196] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Introduction: Interstitial lung diseases (ILDs) represent a heterogeneous group of rare disorders that include more than 200 entities, mostly associated with high mortality. In recent years, the progress regarding the understanding of the pathogenesis of these diseases led to the approval of specific treatments. In ILDs, the presence of comorbidities has a significant impact on the quality of life and the survival of patients and, therefore, their diagnosis and treatment has a pivotal role in management and could improve overall outcome. Areas covered: We discuss key diagnostic issues with regard to the most frequent comorbidities in ILDs. Treatment options are also discussed as the decision to investigate more definitively in order to identify specific comorbidities (including lung cancer, pulmonary hypertension, GE reflux, and obstructive sleep apnoea) is critically dependent upon whether comorbidity-specific treatments are likely to be helpful in individual patients, judged on a case by case basis. Expert opinion: The extent to which clinicians proactively pursue the identification of comorbidities depends on realistic treatment goals in individual patients.
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Affiliation(s)
| | - Maria A Kokosi
- a Interstitial Lung Disease Unit , Royal Brompton Hospital , London , UK
| | - Athol U Wells
- a Interstitial Lung Disease Unit , Royal Brompton Hospital , London , UK
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10
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Reply to 'Cardioimmunology of arrhythmias: the role of autoimmune and inflammatory cardiac channelopathies'. Nat Rev Immunol 2018; 19:65. [PMID: 30552386 DOI: 10.1038/s41577-018-0099-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
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11
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Salama A, Abdullah A, Wahab A, Eigbire G, Hoefen R, Alweis R. Cardiac sarcoidosis and ventricular arrhythmias. A rare association of a rare disease. A retrospective cohort study from the National Inpatient Sample and current evidence for management. Cardiol J 2018; 27:272-277. [PMID: 30234899 DOI: 10.5603/cj.a2018.0104] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2018] [Revised: 09/07/2018] [Accepted: 08/03/2018] [Indexed: 11/25/2022] Open
Abstract
BACKGROUND Sarcoidosis is an increasingly recognized multi-systemic condition. Cardiac sarcoidosis is associated with ventricular arrhythmias and higher mortality rates. Little epidemiological data is available regarding the disease and associated ventricular arrhythmias. METHODS Data from the National Inpatient Sample (NIS) database 2012-2014, were reviewed. Dis-charges associated with sarcoidosis were identified as the target population using relevant ICD-9-CM codes. Primary outcome was a diagnosis of ventricular tachycardia (VT) in the sarcoidosis population. Secondary outcomes include rate of ventricular fibrillation (VF) and cardiac arrest. Subgroup analyses were performed to examine the association of VT with multiple potential confounding clinical variables. RESULTS Of 18,013,878 health encounters, 46,289 (0.26%) subjects had a diagnosis of sarcoidosis. VT and VF were more prevalent among patients with sarcoidosis compared to those without a diagnosis of sarcoidosis (2.29% vs. 1.22%; p < 0.001 and 0.25% vs. 0.21%; p < 0.001, respectively). Sarcoidosis was also associated with a higher prevalence of cardiac arrest (0.72% vs. 0.6%; p < 0.001). In unadjusted analyses, all examined comorbidities were significantly more common in those with sar-coidosis, including diabetes mellitus (31.6% vs. 21.25%; p < 0.001), hypertension (65.2% vs. 51.74%; p < 0.001), chronic kidney disease (21.09% vs. 14.02%; p < 0.001), heart failure (24.87% vs. 15%; p < 0.001) and acute coronary syndrome (4.32% vs. 3.35%; p < 0.001). CONCLUSIONS The present study showed that sarcoidosis was associated with increased rates of ven-tricular tachyarrhythmia, which can affect the overall disease morbidity and mortality.
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Affiliation(s)
- Amr Salama
- Department of Medicine, Unity Hospital, 1555 Long Pond Rd, 14626 Rochester, New York, United States.
| | - Abdullah Abdullah
- Department of Medicine, Unity Hospital, 1555 Long Pond Rd, 14626 Rochester, New York, United States
| | - Abdul Wahab
- Department of Medicine, Unity Hospital, 1555 Long Pond Rd, 14626 Rochester, New York, United States
| | - George Eigbire
- Department of Medicine, Unity Hospital, 1555 Long Pond Rd, 14626 Rochester, New York, United States
| | - Ryan Hoefen
- Department of Medicine, Unity Hospital, 1555 Long Pond Rd, 14626 Rochester, New York, United States.,Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, United States.,Department of Cardiology, Rochester Regional Health, New York, United States
| | - Richard Alweis
- Department of Medicine, Unity Hospital, 1555 Long Pond Rd, 14626 Rochester, New York, United States.,Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, United States.,chool of Health Sciences, Rochester Institute of Technology, New York, United States
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12
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Desai R, Kakumani K, Fong HK, Shah B, Zahid D, Zalavadia D, Doshi R, Goyal H. The burden of cardiac arrhythmias in sarcoidosis: a population-based inpatient analysis. ANNALS OF TRANSLATIONAL MEDICINE 2018; 6:330. [PMID: 30306069 DOI: 10.21037/atm.2018.07.33] [Citation(s) in RCA: 31] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Background Cardiac involvement in the sarcoidosis is known to ensue with diverse clinical forms and its investigation is challenging at times. This article features the under-perceived burden, patterns, and outcomes of different arrhythmias, which may have a prognostic significance in patients with sarcoidosis. Methods We queried the National Inpatient Sample (NIS) for 2010-2014 to recognize sarcoidosis, arrhythmia, and comorbidities affecting hospitalizations. The nationwide estimates were attained using discharge records. We assessed incidence and trends in sarcoidosis-related arrhythmia and consequential inpatient mortality, hospital length of stay (LOS), hospitalization charges and predictors of mortality with multivariate analysis. Results We identified 369,285 sarcoidosis-related hospitalizations. Of these, nearly one-fifth suffered from arrhythmias (n=73,424). The sarcoidosis patients developing arrhythmias were older (61.9 vs. 56.0 years) compared to those without. Males had the higher incidence of arrhythmias compared to females. Atrial fibrillation (Afib) (10.97%) was the most common subtype, followed by ventricular tachycardia (1.97%). There was a rising trend in arrhythmia-related hospital admissions and mortality among sarcoidosis, with Afib incidence displaying the highest increase. Traditional cardiac comorbidities were higher in the sarcoid-arrhythmia group. The arrhythmia group had significantly higher mortality (3.7% vs. 1.5%), mean hospital LOS (6.4 vs. 5.2 days) and hospital charges ($64,118 vs. $41,565) compared to non-arrhythmia group (P<0.001). Incident arrhythmia significantly increased the mortality odds in sarcoidosis (adjusted odds ratio, 2.06). Conclusions The growing trend, deteriorating outcomes and higher mortality associated with sarcoid-related arrhythmias highlight the importance of timely diagnosis and aggressive management in this population.
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Affiliation(s)
- Rupak Desai
- Division of Cardiology, Atlanta VA Medical Center, Decatur, GA, USA
| | | | - Hee Kong Fong
- Department of Internal Medicine, University of Missouri-Columbia, Columbia, MO, USA
| | - Bhrugesh Shah
- Department of Internal Medicine, Staten Island University Hospital Hofstra School of Medicine, Staten Island, NY, USA
| | - Daniyal Zahid
- Department of Internal Medicine, Robert Wood Johnson University Hospital, Hamilton Township, NJ, USA
| | - Dipen Zalavadia
- Department of Internal Medicine, The Wright Center for Graduate Medical Education, Scranton, PA, USA
| | - Rajkumar Doshi
- Department of Internal Medicine, University of Nevada School of Medicine, Reno, NV, USA
| | - Hemant Goyal
- Department of Internal Medicine, Mercer University School of Medicine, Macon, GA, USA
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13
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Patel B, Shah M, Gelaye A, Dusaj R. A complete heart block in a young male: a case report and review of literature of cardiac sarcoidosis. Heart Fail Rev 2018; 22:55-64. [PMID: 27817119 DOI: 10.1007/s10741-016-9585-0] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Cardiac sarcoidosis is one of the uncommon causes of heart failure. Generally, it presents in the form of varying clinical manifestations ranging from asymptomatic to fatal arrhythmias such as ventricular tachycardia and complete heart block. It is difficult to make a diagnosis strictly based on clinical grounds. However, in the setting of extracardiac sarcoidosis and patients presenting with advanced heart block or ventricular arrhythmia, direct cardiac involvement should be suspected. The definitive diagnosis of cardiac sarcoidosis can be made from endomyocardial biopsy, but it is falling out of favor due to patchy myocardial involvement, considerable procedure-related risks, and advancement in additional imaging modalities. Once cardiac sarcoidosis has been diagnosed, management of the disease remains challenging. Steroids are considered the mainstay of therapy, and implantable cardioverter defibrillator therapy can be considered in a selected group of patients at greater risk for malignant ventricular arrhythmias.
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Affiliation(s)
- Brijesh Patel
- Department of Cardiology, Lehigh Valley Hospital, Allentown, PA, USA.
| | - Mahek Shah
- Department of Cardiology, Lehigh Valley Hospital, Allentown, PA, USA
| | - Alehegn Gelaye
- Department of Pulmonary and Critical Care, Providence-Providence Park Hospital, Southfield, MI, USA
| | - Raman Dusaj
- Department of Cardiology, Lehigh Valley Hospital, Allentown, PA, USA
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14
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Atreya AR, Patel M, Sivalingam SK, Stoenescu ML. Isolated cardiac sarcoidosis masquerading as right ventricular outflow tract ventricular tachycardia. BMJ Case Rep 2017; 2017:bcr-2017-220604. [PMID: 28667170 DOI: 10.1136/bcr-2017-220604] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
A 67-year-old man with coronary artery disease (CAD) and left anterior descending artery (LAD) stent presented with symptomatic monomorphic ventricular tachycardia (VT) at a rate of 190 bpm requiring cardioversion. ECG showed left bundle branch block pattern and inferior axis, suggestive of a right ventricular outflow tract (RVOT) focus rather than left ventricular scar due to LAD territory myocardial infarction (MI). Echocardiography showed normal wall motion. Angiography revealed a patent mid-LAD stent. Cardiac MRI with delayed postcontrast sequence revealed several regions of hyperenhancement abnormality within the basal portion of the interventricular septum. Increased metabolic activity on positron emission tomography confirmed active inflammatory sarcoidosis.Although VTs in patients with prior CAD are likely to be related to either scar or ischaemia, alternative diagnoses (eg, infiltrative disorders, RVOT-VT, arrhythmogenic right ventricular cardiomyopathy) should be considered in patients with an apparent right ventricular focus on ECG.
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Affiliation(s)
- Auras R Atreya
- University of Massachusetts Medical School-Baystate, Springfield, Massachusetts, USA
| | - Mitkumar Patel
- University of Massachusetts Medical School-Baystate, Springfield, Massachusetts, USA
| | - Senthil K Sivalingam
- University of Massachusetts Medical School-Baystate, Springfield, Massachusetts, USA
| | - Mathias L Stoenescu
- University of Massachusetts Medical School-Baystate, Springfield, Massachusetts, USA
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15
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Margaritopoulos GA, Antoniou KM, Wells AU. Comorbidities in interstitial lung diseases. Eur Respir Rev 2017; 26:160027. [PMID: 28049126 PMCID: PMC9488735 DOI: 10.1183/16000617.0027-2016] [Citation(s) in RCA: 62] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2016] [Accepted: 04/16/2016] [Indexed: 12/20/2022] Open
Abstract
Fibrosing lung disorders include a large number of diseases with diverse behaviour. Patients can die because of the progression of their illness, remain stable or even improve after appropriate treatment has been instituted. Comorbidities, such as acute and chronic infection, gastro-oesophageal reflux, pulmonary hypertension, lung cancer, cardiovascular diseases, and obstructive sleep apnoea, can pre-exist or develop at any time during the course of the disease and, if unidentified and untreated, may impair quality of life, impact upon the respiratory status of the patients, and ultimately lead to disease progression and death. Therefore, early identification and accurate treatment of comorbidities is essential.
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Affiliation(s)
| | - Katerina M Antoniou
- Interstitial Lung Disease Unit, University Hospital of Heraklion, Heraklion, Greece
| | - Athol U Wells
- Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK
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16
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Stephenson E, Savvatis K, Mohiddin SA, Marelli-Berg FM. T-cell immunity in myocardial inflammation: pathogenic role and therapeutic manipulation. Br J Pharmacol 2016; 174:3914-3925. [PMID: 27590129 DOI: 10.1111/bph.13613] [Citation(s) in RCA: 46] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2016] [Revised: 08/11/2016] [Accepted: 08/16/2016] [Indexed: 12/13/2022] Open
Abstract
T-cell-mediated immunity has been linked not only to a variety of heart diseases, including classic inflammatory diseases such as myocarditis and post-myocardial infarction (Dressler's) syndrome, but also to conditions without an obvious inflammatory component such as idiopathic dilated cardiomyopathy and hypertensive cardiomyopathy. It has been recently proposed that in all these conditions, the heart becomes the focus of T-cell-mediated autoimmune inflammation following ischaemic or infectious injury. For example, in acute myocarditis, an inflammatory disease of heart muscle, T-cell responses are thought to arise as a consequence of a viral infection. In a number of patients, persistent T-cell-mediated responses in acute viral myocarditis can lead to autoimmunity and chronic cardiac inflammation resulting in dilated cardiomyopathy. In spite of the major progress made in understanding the mechanisms of pathogenic T-cell responses, effective and safe therapeutic targeting of the immune system in chronic inflammatory diseases of the heart has not yet been developed due to the lack of specific diagnostic and prognostic biomarkers at an early stage. This has also prevented the identification of targets for patient-tailored immunomodulatory therapies that are both disease- and organ-selective. In this review, we discuss current knowledge of the development and functional characteristics of pathogenic T-cell-mediated immune responses in the heart, and, in particular, in myocarditis, as well as recent advances in experimental models which have the potential to translate into heart-selective immunomodulation. LINKED ARTICLES This article is part of a themed section on Targeting Inflammation to Reduce Cardiovascular Disease Risk. To view the other articles in this section visit http://onlinelibrary.wiley.com/doi/10.1111/bph.v174.22/issuetoc and http://onlinelibrary.wiley.com/doi/10.1111/bcp.v82.4/issuetoc.
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Affiliation(s)
- E Stephenson
- William Harvey Research Institute, London, UK.,Barts and The London School of Medicine, London, UK
| | - K Savvatis
- William Harvey Research Institute, London, UK.,Barts and The London School of Medicine, London, UK.,Department of Cardiology, Barts Heart Centre, St. Bartholomew NHS Trust, London, UK
| | - S A Mohiddin
- William Harvey Research Institute, London, UK.,Barts and The London School of Medicine, London, UK.,Department of Cardiology, Barts Heart Centre, St. Bartholomew NHS Trust, London, UK
| | - F M Marelli-Berg
- William Harvey Research Institute, London, UK.,Barts and The London School of Medicine, London, UK
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Ekström K, Lehtonen J, Hänninen H, Kandolin R, Kivistö S, Kupari M. Magnetic Resonance Imaging as a Predictor of Survival Free of Life-Threatening Arrhythmias and Transplantation in Cardiac Sarcoidosis. J Am Heart Assoc 2016; 5:e003040. [PMID: 27139734 PMCID: PMC4889179 DOI: 10.1161/jaha.115.003040] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2015] [Accepted: 03/07/2016] [Indexed: 12/12/2022]
Abstract
BACKGROUND Cardiac magnetic resonance imaging has a key role in today's diagnosis of cardiac sarcoidosis. We set out to investigate whether cardiac magnetic resonance imaging also helps predict outcome in cardiac sarcoidosis. METHODS AND RESULTS Our work involved 59 patients with cardiac sarcoidosis (38 female, mean age 46±10 years) seen at our hospital since February 2004 and followed up after contrast-enhanced cardiac magnetic resonance imaging. The extent of myocardial late gadolinium enhancement (measured as percentage of left ventricular mass), the volumes and ejection fractions of the left and right ventricles, and the thickness of the basal interventricular septum were determined and analyzed for prognostic significance. By April 2015, 23 patients had reached the study's end point, consisting of a composite of cardiac death (n=3), cardiac transplantation (n=1), and occurrence of life-threatening ventricular tachyarrhythmias (n=19; ventricular fibrillation in 5 and sustained ventricular tachycardia in 14 patients). In univariate analysis, myocardial extent of late gadolinium enhancement predicted event-free survival, as did scar-like thinning (<4 mm) of the basal interventricular septum and the ejection fraction of the right ventricle (P<0.05 for all). In multivariate Cox regression analysis, extent of late gadolinium enhancement was the only independent predictor of outcome events on cardiac magnetic resonance imaging, with a hazard ratio of 2.22 per tertile (95% CI 1.07-4.59). An extent of late gadolinium enhancement >22% (third tertile) had positive and negative predictive values for serious cardiac events of 75% and 76%, respectively. CONCLUSIONS Findings on cardiac magnetic resonance imaging and the extent of myocardial late gadolinium enhancement in particular help predict serious cardiac events in cardiac sarcoidosis.
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Affiliation(s)
- Kaj Ekström
- The Heart and Lung Center, Radiology, Helsinki University Central Hospital, Helsinki, Finland
| | - Jukka Lehtonen
- The Heart and Lung Center, Radiology, Helsinki University Central Hospital, Helsinki, Finland
| | - Helena Hänninen
- The Heart and Lung Center, Radiology, Helsinki University Central Hospital, Helsinki, Finland
| | - Riina Kandolin
- The Heart and Lung Center, Radiology, Helsinki University Central Hospital, Helsinki, Finland
| | - Sari Kivistö
- HUS Medical Imaging Center, Radiology, Helsinki University Central Hospital, Helsinki, Finland
| | - Markku Kupari
- The Heart and Lung Center, Radiology, Helsinki University Central Hospital, Helsinki, Finland
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18
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Dubrey SW, Sharma R, Underwood R, Mittal T. Cardiac sarcoidosis: diagnosis and management. Postgrad Med J 2015; 91:384-94. [PMID: 26130811 DOI: 10.1136/postgradmedj-2014-133219] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2014] [Accepted: 06/12/2015] [Indexed: 12/19/2022]
Abstract
Cardiac sarcoidosis is one of the most serious and unpredictable aspects of this disease state. Heart involvement frequently presents with arrhythmias or conduction disease, although myocardial infiltration resulting in congestive heart failure may also occur. The prognosis in cardiac sarcoidosis is highly variable, which relates to the heterogeneous nature of heart involvement and marked differences between racial groups. Electrocardiography and echocardiography often provide the first clue to the diagnosis, but advanced imaging studies using positron emission tomography and MRI, in combination with nuclear isotope perfusion scanning are now essential to the diagnosis and management of this condition. The identification of clinically occult cardiac sarcoidosis and the management of isolated and/or asymptomatic heart involvement remain both challenging and contentious. Corticosteroids remain the first treatment choice with the later substitution of immunosuppressive and steroid-sparing therapies. Heart transplantation is an unusual outcome, but when performed, the results are comparable or better than heart transplantation for other disease states. We review the epidemiology, developments in diagnostic techniques and the management of cardiac sarcoidosis.
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Affiliation(s)
- S W Dubrey
- Department of Cardiology, Hillingdon Hospital, Uxbridge, UK
| | - R Sharma
- Department of Cardiology, The Royal Brompton Hospital, London, UK
| | - R Underwood
- Department of Radiology, Harefield Hospital, Harefield, UK
| | - T Mittal
- Department of Radiology, Harefield Hospital, Harefield, UK
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19
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Değirmenci H, Demirelli S, Arısoy A, Ermiş E, Araz Ö, Bakırcı EM, Hamur H, Büyüklü M, Topal E. Myocardial deformation and total atrial conduction time in the prediction of cardiac involvement in patients with pulmonary sarcoidosis. CLINICAL RESPIRATORY JOURNAL 2015; 11:68-77. [DOI: 10.1111/crj.12307] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/22/2014] [Revised: 03/23/2015] [Accepted: 04/21/2015] [Indexed: 11/30/2022]
Affiliation(s)
- Hüsnü Değirmenci
- Faculty of Medicine; Cardiology; Erzincan University; Erzincan Turkey
| | - Selami Demirelli
- Department of Cardiology; Education and Research Hospital; Erzurum Turkey
| | - Arif Arısoy
- Faculty of Medicine; Cardiology; Gaziosmanpasa University; Tokat Turkey
| | - Emrah Ermiş
- Department of Cardiology; Education and Research Hospital; Erzurum Turkey
| | - Ömer Araz
- Faculty of Medicine; Pulmonary of Disease; Ataturk University; Erzurum Turkey
| | | | - Hikmet Hamur
- Faculty of Medicine; Cardiology; Erzincan University; Erzincan Turkey
| | - Mutlu Büyüklü
- Faculty of Medicine; Cardiology; Erzincan University; Erzincan Turkey
| | - Ergün Topal
- Faculty of Medicine; Cardiology; Erzincan University; Erzincan Turkey
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20
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Sensoy B, Ozeke O, Canpolat U, Colak A, Grbovic E, Cay S, Topaloglu S, Aras D, Aydogdu S. Phase-4 paroxysmal atrioventricular block in sarcoidosis. Herz 2015; 40:321-324. [PMID: 24297398 DOI: 10.1007/s00059-013-4002-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2013] [Revised: 09/16/2013] [Accepted: 09/26/2013] [Indexed: 11/28/2022]
Affiliation(s)
- B Sensoy
- Department of Cardiology, Turkiye Yuksek Ihtisas Hospital, 06100, Ankara, Turkey
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21
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Kron J, Sauer W, Mueller G, Schuller J, Bogun F, Sarsam S, Rosenfeld L, Mitiku TY, Cooper JM, Mehta D, Greenspon AJ, Ortman M, Delurgio DB, Valadri R, Narasimhan C, Swapna N, Singh JP, Danik S, Markowitz SM, Almquist AK, Krahn AD, Wolfe LG, Feinstein S, Ellenbogen KA, Crawford T. Outcomes of patients with definite and suspected isolated cardiac sarcoidosis treated with an implantable cardiac defibrillator. J Interv Card Electrophysiol 2015; 43:55-64. [PMID: 25676929 DOI: 10.1007/s10840-015-9978-3] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2014] [Accepted: 01/20/2015] [Indexed: 10/24/2022]
Abstract
PURPOSE Cardiac sarcoidosis (CS) patients are at increased risk for sudden death. Isolated CS is rare and can be difficult to diagnose. METHODS In this multicenter retrospective review, patients with CS and an implantable cardiac defibrillator (ICD) were identified. RESULTS Of 235 patients with CS and ICD, 13 (5.5 %) had isolated CS, including 7 (3.0 %) with definite isolated CS (biopsy or necropsy-proven) and 6 (2.6 %) with suspected isolated CS based on a constellation of clinical, ECG, and imaging findings. Among 13 patients with isolated CS, 10 (76.9 %) were male, mean age was 53.8 ± 7.6 years, and mean left ventricular ejection fraction was 38.3 ± 16.5. Diagnosis was made by cardiac magnetic resonance (CMR) (n = 2), biopsy (n = 3), CMR and biopsy (n = 2), CMR and positron emission tomography (PET) (n = 2), PET (n = 1), late enhanced cardiac CT (n = 1), pathology at heart transplant (n = 1), and autopsy (n = 1). Eight of 13 (61.5 %) patients with isolated CS had a secondary prevention indication (VT in 6 and VF in 2) vs. 80 of 222 (36.0 %) with sarcoidosis in other organs (p = 0.04). Over a mean of 4.2 years, 9 of 13 (69.2 %) patients with isolated CS received appropriate ICD therapy, including anti-tachycardia pacing (ATP) and/or shock, compared with 75 of 222 (33.8 %) patients with cardiac and extracardiac sarcoidosis (p = 0.0150). Six of 7 (85.7 %) patients with definite isolated CS received appropriate ICD intervention, compared with 78 of 228 patients (34.2 %) without definite isolated CS (p = 0.0192.) CONCLUSIONS In this retrospective study, patients with isolated CS had very high rates of appropriate ICD therapy. Prospective, long-term follow-up of consecutive patients with isolated CS is needed to determine the true natural history and rates of ventricular arrhythmias in this rare and difficult-to-diagnose disease.
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Affiliation(s)
- Jordana Kron
- Department of Cardiac Electrophysiology, Virginia Commonwealth University, P.O. Box 980053, Richmond, VA, 23298-0053, USA,
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Wicks EC, Menezes LJ, Elliott PM. Improving the diagnostic accuracy for detecting cardiac sarcoidosis. Expert Rev Cardiovasc Ther 2015; 13:223-36. [DOI: 10.1586/14779072.2015.1001367] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
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23
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Saremi F, Saremi A, Hassani C, Cen S, Lin L, Ng B, Shahriarian S. Computed Tomographic Diagnosis of Myocardial Fat Deposits in Sarcoidosis. J Comput Assist Tomogr 2015; 39:578-83. [DOI: 10.1097/rct.0000000000000235] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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24
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Şentürk A, Maraş Y, Argüder E, Karalezli A, Hasanoğlu HC, Öğüt T, Baştuğ S, Karabekir E. What type of different clinical manifestations can cardiac sarcoidosis present? Rheumatol Int 2014; 35:1103-6. [PMID: 25429793 DOI: 10.1007/s00296-014-3183-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2014] [Accepted: 11/18/2014] [Indexed: 11/25/2022]
Abstract
Cardiac sarcoidosis is an infiltrative, granulomatous inflammatory disease of the myocardium. Generally, it can be difficult to diagnose cardiac sarcoidosis clinically because of the non-specific nature of its clinical manifestations. This property can be based on the presence of any clinical evidence of sarcoidosis in the other organs. We present two cases of cardiac sarcoidosis so as to demonstrate its different clinical manifestations. The first patient displayed no cardiac symptoms; the electrocardiogram showed an incidental right bundle branch block. Her cardiac magnetic resonance imaging (CMRI) revealed late-phase opaque material enhancement involving the inferior and inferoseptal segment of the left ventricle. The second patient was severely symptomatic in terms of cardiac involvement, and a transthoracic echocardiogram revealed global hypokinesia and septal brightness; his ejection fraction decreased to 45%. These cases highlighted the challenges encountered in the diagnosis and treatment of cardiac sarcoidosis. CMRI should be considered in all patients who have suspected findings for cardiac involvement.
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Affiliation(s)
- Ayşegül Şentürk
- Department of Chest Disease, Atatürk Training and Research Hospital, Ankara, Turkey
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25
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26
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Sisakian H. Cardiomyopathies: Evolution of pathogenesis concepts and potential for new therapies. World J Cardiol 2014; 6:478-494. [PMID: 24976920 PMCID: PMC4072838 DOI: 10.4330/wjc.v6.i6.478] [Citation(s) in RCA: 61] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/09/2014] [Revised: 03/06/2014] [Accepted: 03/14/2014] [Indexed: 02/06/2023] Open
Abstract
Cardiomyopathies are defined as diseases of the myocardium with associated structural and functional abnormalities. Knowledge of these pathologies for a long period was not clear in clinical practice due to uncertainties regarding definition, classification and clinical diagnosis. In recent decades, major advances have been made in the understanding of the molecular and genetic issues, pathophysiology, and clinical and radiological assessment of the diseases. Progress has been made also in management of several types of cardiomyopathy. Advances in the understanding of these diseases show that cardiomyopathies represent complex entities. Here, special attention is given to evolution of classification of cardiomyopathies, with the aim of assisting clinicians to look beyond schematic diagnostic labels in order to achieve more specific diagnosis. Knowledge of the genotype of cardiomyopathies has changed the pathophysiological understanding of their etiology and clinical course, and has become more important in clinical practice for diagnosis and prevention of cardiomyopathies. New approaches for clinical and prognostic assessment are provided based on contemporary molecular mechanisms of contribution in the pathogenesis of cardiomyopathies. The genotype-phenotype complex approach for assessment improves the clinical evaluation and management strategies of these pathologies. The review covers also the important role of imaging methods, particularly echocardiography, and cardiac magnetic resonance imaging in the evaluation of different types of cardiomyopathies. In summary, this review provides complex presentation of current state of cardiomyopathies from genetics to management aspects for cardiovascular specialists.
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27
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WILLNER JONATHANM, VILES-GONZALEZ JUANF, COFFEY JAMESO, MORGENTHAU ADAMS, MEHTA DAVENDRA. Catheter Ablation of Atrial Arrhythmias in Cardiac Sarcoidosis. J Cardiovasc Electrophysiol 2014; 25:958-963. [DOI: 10.1111/jce.12424] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2014] [Revised: 03/18/2014] [Accepted: 03/25/2014] [Indexed: 10/25/2022]
Affiliation(s)
- JONATHAN M. WILLNER
- Divisions of Cardiology and Pulmonary Medicine; Mount Sinai Medical Center and Icahn School of Medicine at Mount Sinai; New York New York USA
| | - JUAN F. VILES-GONZALEZ
- Division of Cardiology; University of Miami Medical Center and Miami School of Medicine; Miami Florida
| | - JAMES O. COFFEY
- Division of Cardiology; University of Miami Medical Center and Miami School of Medicine; Miami Florida
| | - ADAM S. MORGENTHAU
- Divisions of Cardiology and Pulmonary Medicine; Mount Sinai Medical Center and Icahn School of Medicine at Mount Sinai; New York New York USA
| | - DAVENDRA MEHTA
- Divisions of Cardiology and Pulmonary Medicine; Mount Sinai Medical Center and Icahn School of Medicine at Mount Sinai; New York New York USA
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28
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Osborne MT, Hulten EA, Singh A, Waller AH, Bittencourt MS, Stewart GC, Hainer J, Murthy VL, Skali H, Dorbala S, Di Carli MF, Blankstein R. Reduction in ¹⁸F-fluorodeoxyglucose uptake on serial cardiac positron emission tomography is associated with improved left ventricular ejection fraction in patients with cardiac sarcoidosis. J Nucl Cardiol 2014; 21:166-74. [PMID: 24307261 DOI: 10.1007/s12350-013-9828-6] [Citation(s) in RCA: 211] [Impact Index Per Article: 19.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2013] [Accepted: 11/11/2013] [Indexed: 10/26/2022]
Abstract
BACKGROUND Cardiac positron emission tomography (PET) using (18)F-fluorodeoxyglucose (FDG) has been used to diagnose and monitor cardiac sarcoidosis (CS). It is not known whether a reduction in myocardial inflammation, as measured by FDG uptake, is associated with improvement in LV ejection fraction (EF). METHODS For 23 patients with CS followed by a total of 90 serial PET exams (median 4 per patient), two physicians blinded to EF quantified the maximum of standardized uptake value (SUV) and volume of inflamed tissue above two distinct thresholds to assess the intensity and extent of FDG uptake on each study. Using gated (82)Rubidium rest myocardial perfusion images, EF was measured blinded to all clinical and FDG data. To account for clustering and differences in scan frequency, a longitudinal mixed effects model was used to evaluate the relationship between FDG uptake and changes in EF on interval scans. RESULTS Among 23 patients with serial PET exams (mean age 49, 74% male, mean baseline EF 43% ± 13%), the median time between the first and last scan was 2.0 years. Overall, 91% were treated with corticosteroids, 78% with ACE/ARB, 83% with beta-blockers, and 83% had ICDs. Longitudinal regression demonstrated a significant inverse linear relationship between maximum SUV and EF with an expected increase in EF of 7.9% per SUV reduction of 10 g·mL(-1) (P = .008). Likewise, in an analysis based on volume, there was an increase in EF of 2.1% per 100 cm(3) decrease in volume of inflamed tissue using a threshold of 2.7 g·mL(-1) (P = .028) and an increase in EF of 3.8% per 100 cm(3) decrease (P = .022) using a SUV threshold of 4.1 g·mL(-1). CONCLUSIONS In a longitudinal cohort of CS patients, a reduction in the intensity and extent of myocardial inflammation on FDG PET is associated with improvement in EF. These data suggest serial PET scanning may help guide titration of immunosuppressive therapy to improve or prevent heart failure in CS.
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Affiliation(s)
- Michael T Osborne
- Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA
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Adamson P, Melton I, O'Donnell J, MacDonald S, Crozier I. Cardiac sarcoidosis: the Christchurch experience. Intern Med J 2014; 44:70-6. [DOI: 10.1111/imj.12314] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2013] [Accepted: 10/16/2013] [Indexed: 01/21/2023]
Affiliation(s)
- P. Adamson
- Department of Cardiology; Christchurch Hospital; Christchurch New Zealand
| | - I. Melton
- Department of Cardiology; Christchurch Hospital; Christchurch New Zealand
| | - J. O'Donnell
- Department of Immunology; Christchurch Hospital; Christchurch New Zealand
| | - S. MacDonald
- Department of Radiology; Christchurch Hospital; Christchurch New Zealand
| | - I. Crozier
- Department of Cardiology; Christchurch Hospital; Christchurch New Zealand
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Aljaroudi WA, Flamm SD, Saliba W, Wilkoff BL, Kwon D. Role of CMR imaging in risk stratification for sudden cardiac death. JACC Cardiovasc Imaging 2013; 6:392-406. [PMID: 23473115 DOI: 10.1016/j.jcmg.2012.11.011] [Citation(s) in RCA: 52] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2012] [Revised: 11/19/2012] [Accepted: 11/26/2012] [Indexed: 12/28/2022]
Abstract
Left ventricular ejection fraction as determined by echocardiography has a limited sensitivity in predicting risk for sudden cardiac death (SCD). Subsequent efforts to improve cost-effectiveness of device implantation and identify a better risk-stratifying tool have been quite desirable. The presence of scar and myocardial tissue heterogeneity has been linked to ventricular arrhythmia, which is believed to be the major cause of SCD. Cardiac magnetic resonance is a noninvasive imaging modality that visualizes and quantifies scar, with growing evidence delineating its additive value in identifying patients at higher risk for SCD.
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MESH Headings
- Arrhythmias, Cardiac/diagnosis
- Arrhythmias, Cardiac/etiology
- Arrhythmias, Cardiac/pathology
- Arrhythmias, Cardiac/physiopathology
- Arrhythmias, Cardiac/therapy
- Cardiomyopathies/complications
- Cardiomyopathies/diagnosis
- Cardiomyopathies/pathology
- Cardiomyopathies/physiopathology
- Death, Sudden, Cardiac/etiology
- Death, Sudden, Cardiac/prevention & control
- Defibrillators, Implantable
- Electric Countershock/instrumentation
- Humans
- Magnetic Resonance Imaging
- Myocardium/pathology
- Predictive Value of Tests
- Prognosis
- Risk Assessment
- Risk Factors
- Stroke Volume
- Ventricular Function, Left
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Affiliation(s)
- Wael A Aljaroudi
- Heart and Vascular Institute, Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio, USA.
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31
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Reduction in ¹⁸F-fluorodeoxyglucose uptake on serial cardiac positron emission tomography is associated with improved left ventricular ejection fraction in patients with cardiac sarcoidosis. J Nucl Cardiol 2013. [PMID: 24307261 DOI: 10.1007/s12350-013-98286] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
BACKGROUND Cardiac positron emission tomography (PET) using (18)F-fluorodeoxyglucose (FDG) has been used to diagnose and monitor cardiac sarcoidosis (CS). It is not known whether a reduction in myocardial inflammation, as measured by FDG uptake, is associated with improvement in LV ejection fraction (EF). METHODS For 23 patients with CS followed by a total of 90 serial PET exams (median 4 per patient), two physicians blinded to EF quantified the maximum of standardized uptake value (SUV) and volume of inflamed tissue above two distinct thresholds to assess the intensity and extent of FDG uptake on each study. Using gated (82)Rubidium rest myocardial perfusion images, EF was measured blinded to all clinical and FDG data. To account for clustering and differences in scan frequency, a longitudinal mixed effects model was used to evaluate the relationship between FDG uptake and changes in EF on interval scans. RESULTS Among 23 patients with serial PET exams (mean age 49, 74% male, mean baseline EF 43% ± 13%), the median time between the first and last scan was 2.0 years. Overall, 91% were treated with corticosteroids, 78% with ACE/ARB, 83% with beta-blockers, and 83% had ICDs. Longitudinal regression demonstrated a significant inverse linear relationship between maximum SUV and EF with an expected increase in EF of 7.9% per SUV reduction of 10 g·mL(-1) (P = .008). Likewise, in an analysis based on volume, there was an increase in EF of 2.1% per 100 cm(3) decrease in volume of inflamed tissue using a threshold of 2.7 g·mL(-1) (P = .028) and an increase in EF of 3.8% per 100 cm(3) decrease (P = .022) using a SUV threshold of 4.1 g·mL(-1). CONCLUSIONS In a longitudinal cohort of CS patients, a reduction in the intensity and extent of myocardial inflammation on FDG PET is associated with improvement in EF. These data suggest serial PET scanning may help guide titration of immunosuppressive therapy to improve or prevent heart failure in CS.
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32
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Umazume A, Kezuka T, Okunuki Y, Ooshita M, Usui Y, Hirano M, Yamashina A, Goto H. Prediction of severe cardiac involvement by fundus lesion in sarcoidosis. Jpn J Ophthalmol 2013; 58:81-5. [PMID: 24212542 DOI: 10.1007/s10384-013-0288-y] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/31/2013] [Accepted: 09/26/2013] [Indexed: 11/29/2022]
Abstract
PURPOSE We examined the relation between ocular sarcoidosis and severe cardiac sarcoidosis necessitating pacemaker implantation. METHODS In this retrospective, observational, cross-sectional study, we reviewed the clinical records of 108 patients diagnosed with ocular sarcoidosis based on new diagnostic criteria established in Japan. We examined and compared the relationship between fundus findings of ocular sarcoidosis and severe cardiac sarcoidosis necessitating pacemaker implantation. RESULTS Of 108 patients with ocular sarcoidosis, seven patients (6.5 %) with median age of 61 years (interquartile range 59-63 years) also had severe heart disease leading to implantation of a pacemaker. Median duration of ocular symptoms was 4.5 years (interquartile range 3.6-7.8 years). Of seven patients with severe cardiac involvement, six had multiple peripheral chorioretinal atrophic lesions (MPCAL), two had nodules in the angle and tent-like peripheral anterior synechia, and two had snowball vitreous opacity. The prevalence of atrophic MPCAL lesions was significantly higher than that of other ocular findings (P < 0.05). CONCLUSIONS In patients with concurrent ocular sarcoidosis and severe cardiac sarcoidosis, MPCAL atrophic lesions were observed significantly more frequently, suggesting that severe cardiac involvement may be predicted by specific fundus lesions.
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Affiliation(s)
- Akihiko Umazume
- Department of Ophthalmology, Tokyo Medical University, 6-7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan
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Nery PB, Mc Ardle BA, Redpath CJ, Leung E, Lemery R, Dekemp R, Yang J, Keren A, Beanlands RS, Birnie DH. Prevalence of cardiac sarcoidosis in patients presenting with monomorphic ventricular tachycardia. PACING AND CLINICAL ELECTROPHYSIOLOGY: PACE 2013; 37:364-74. [PMID: 24102263 DOI: 10.1111/pace.12277] [Citation(s) in RCA: 67] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 04/28/2013] [Revised: 07/23/2013] [Accepted: 07/30/2013] [Indexed: 11/29/2022]
Abstract
INTRODUCTION Sarcoidosis is a granulomatous disease of unknown etiology, which involves the heart in 5-25% of cases. Although ventricular tachycardia (VT) has been reported as the first presentation of sarcoidosis, its prevalence has not previously been investigated. In this prospective study, we sought to systematically investigate the prevalence of cardiac sarcoidosis (CS) in patients presenting with monomorphic VT (MMVT) and no previous history of sarcoidosis. METHODS Consecutive patients presenting with MMVT to a tertiary care center were screened for inclusion. Patients with idiopathic VT, VT secondary to coronary artery disease, or prior diagnosis of sarcoidosis were excluded. Included patients underwent F-18-fluorodeoxyglucose positron emission tomography (PET) scan. In subjects with PET scanning suggestive of active myocardial inflammation, histological diagnosis was confirmed through extracardiac or endomyocardial biopsy (EMB). RESULTS A total of 182 patients presented to our institution with VT between February 2010 and September 2012 and 14 subjects met inclusion criteria. Within this group, six of 14 (42%) patients had abnormal PET scans suggesting active myocardial inflammation. Four of the six patients had tissue biopsies that were diagnostic of sarcoidosis; the remaining two patients had guided EMB indicating nonspecific myocarditis. Atrioventricular block was observed in three of four (75%) patients with CS and none in 10 of the others (P = 0.022). Three of the four patients had pulmonary sarcoidosis and one patient had isolated CS. All four patients were treated with corticosteroids. CONCLUSION In this prospective study, four of 14 (28%) patients presenting with MMVT (without idiopathic VT, ischemic VT, or known sarcoidosis) had CS as the underlying etiology. Clinicians should consider screening for CS in patients with unexplained MMVT.
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Affiliation(s)
- Pablo B Nery
- Division of Cardiology, Department of Medicine, University of Ottawa Heart Institute, Ottawa, Ontario, Canada
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Mc Ardle BA, Birnie DH, Klein R, de Kemp RA, Leung E, Renaud J, DaSilva J, Wells GA, Beanlands RS, Nery PB. Is There an Association Between Clinical Presentation and the Location and Extent of Myocardial Involvement of Cardiac Sarcoidosis as Assessed by
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F- Fluorodoexyglucose Positron Emission Tomography? Circ Cardiovasc Imaging 2013; 6:617-26. [DOI: 10.1161/circimaging.112.000289] [Citation(s) in RCA: 76] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Affiliation(s)
- Brian A. Mc Ardle
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - David H. Birnie
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - Ran Klein
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - Rob A. de Kemp
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - Eugene Leung
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - Jennifer Renaud
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - Jean DaSilva
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - George A. Wells
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - Rob S. Beanlands
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
| | - Pablo B. Nery
- From the Arrhythmia Service (D.H.B., P.B.N), Cardiac Research and Methods Center (G.A.W.), and the National Cardiac PET Centre, Division of Cardiology, Department of Medicine (B.A.M., R.K., R.A.d.K., J.R., J.D., R.S.B.), University of Ottawa Heart Institute; and the Division of Nuclear Medicine, Department of Medicine, The Ottawa Hospital and University of Ottawa (E.L.), Ottawa, Ontario, Canada
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Mc Ardle BA, Leung E, Ohira H, Cocker MS, deKemp RA, DaSilva J, Birnie D, Beanlands RS, Nery PB. The role of F(18)-fluorodeoxyglucose positron emission tomography in guiding diagnosis and management in patients with known or suspected cardiac sarcoidosis. J Nucl Cardiol 2013; 20:297-306. [PMID: 23288545 DOI: 10.1007/s12350-012-9668-9] [Citation(s) in RCA: 56] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2012] [Accepted: 12/09/2012] [Indexed: 11/30/2022]
Abstract
Cardiac sarcoidosis (CS) has gained significant interest in recent years with the emergence of advanced imaging modalities such as MRI and F(18)-fluorodeoxyglucose-positron emission tomography (FDG-PET) as modalities to aid in the diagnosis of this condition. CS remains a difficult condition to diagnose, particularly in cases of isolated cardiac involvement and it can present with a broad spectrum of clinical syndromes. Furthermore, the appropriate management of these patients remains controversial. FDG-PET has a potential role not only in diagnosis of CS but also in directing further therapies, facilitating the decision to start immunosuppression and monitoring the response to it. In this article, we discuss when to consider FDG-PET, outline the current optimal patient preparation and scanning protocols and then, using case examples, discuss the use of FDG-PET in follow-up of patients with known or suspected CS. We also outline how PET can influence management decisions in these patients.
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Affiliation(s)
- B A Mc Ardle
- Division of Cardiology, Department of Medicine, Arrhythmia Service, University of Ottawa Heart Institute, Ottawa, ON, Canada
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Srivatsa UN, Rogers J. Sarcoidosis and atrial fibrillation: a rare association and interlink with inflammation. Indian Pacing Electrophysiol J 2012; 12:290-1. [PMID: 23233763 PMCID: PMC3513243 DOI: 10.1016/s0972-6292(16)30569-1] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
We report a case of sarcoidosis presenting initially as atrial fibrillation(AF). His response to anti-arrhythmic treatment strategy was suboptimal. On initiation of immunosuppressive therapy, AF was better controlled. This interesting case highlights a likely link between inflammation and pathogenesis of atrial fibrillation.
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Affiliation(s)
- Uma N Srivatsa
- UC Davis Medical Center, 4860 Y street, Ste 2820, Sacramento, CA
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Cardiac Sarcoidosis - Arrhythmias, Inflammation and Anti-inflammatory Drug Therapy. Indian Pacing Electrophysiol J 2012; 12:234-6. [PMID: 23233756 PMCID: PMC3513237 DOI: 10.1016/s0972-6292(16)30562-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023] Open
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