Isolated cardiac sarcoidosis with rapid progressive 'malignant' arrhythmia.
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ABSTRACT:
SUBMITTER: Tang Y
PROVIDER: S-EPMC9949704 | biostudies-literature | 2023 Feb
REPOSITORIES: biostudies-literature
Publications
Isolated cardiac sarcoidosis with rapid progressive 'malignant' arrhythmia.
Tang Yun Y Wang Jia-Xin JX Chen Xiu-Yu XY Wang Hong-Yue HY Zhao Shi-Hua SH
European heart journal. Case reports 20230201 2
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| S-EPMC9333370 | 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.
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| S-EPMC5179170 | biostudies-literature
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| S-EPMC8370865 | 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:BackgroundCardiac sarcoidosis (CS) accounts for a substantial morbidity and mortality. Early recognition of CS is important to prevent such detrimental consequences. A definite diagnosis of cardiac sarcoidosis remains challenging. Even after the diagnosis of CS is established, the appropriate dose and duration of corticosteroids in the treatment of CS have not been well-defined.Case summaryIn this report, we discuss a case of a 50-year-old man who presented with recurrent syncope. Electrocardiogram revealed sinus rhythm with left bundle branch block. Telemetry captured high-grade atrioventricular block. Coronary angiogram showed no coronary artery disease. Left ventriculography revealed left ventricular ejection fraction (LVEF) of 35-40%. A dual-chamber pacemaker was implanted. Cardiac magnetic resonance revealed mid-myocardial scarring suggestive of sarcoidosis. Computed tomography of the chest showed lymphadenopathy. Transbronchial biopsy was unrevealing; however, mediastinoscopy and lymph node biopsy showed non-caseating granulomas diagnostic of sarcoidosis. He became pacemaker dependent as noted in outpatient pacemaker interrogations. A biventricular implantable cardioverter-defibrillator upgrade was performed for primary prevention of sudden cardiac death. He was started on prednisone taper over the course of 6 months. After 1-year, his LVEF improved to 55% and native atrioventricular (AV) conduction had recovered as noted in outpatient device interrogations.DiscussionThis case highlights the importance to include CS in the differential diagnosis of a young patient with conduction system disease and non-ischaemic cardiomyopathy for appropriate treatment. Patients with left ventricular systolic dysfunction and AV nodal disease could potentially benefit from a slow prednisone taper over the course of 6 months.
| S-EPMC6426016 | biostudies-literature
Project description:There is accumulating evidence for the existence of a phenotype of isolated cardiac sarcoidosis (ICS), or sarcoidosis that only involves the heart. In the absence of biopsy-confirmed cardiac sarcoidosis (CS), existing diagnostic criteria require the presence of extra-cardiac sarcoidosis as an inclusion criterion for the diagnosis of CS. Consequently, in the absence of a positive endomyocardial biopsy, ICS is not diagnosable by current guidelines. Therefore, there is uncertainty regarding the epidemiology, pathobiology, clinical characteristics, prognosis, and optimal treatment of ICS. This review will summarize the available data related to the prevalence and prognosis of ICS and will discuss challenges surrounding the diagnosis and management of this under-recognized entity.
| S-EPMC5540795 | biostudies-literature
Project description:Background Cardiac sarcoidosis is a chronic, inflammatory disease that can affect the heart and often results in heart failure and lethal arrhythmias. A multimodality imaging approach without endomyocardial biopsy allows for the diagnosis of isolated cardiac sarcoidosis. Coronary vasomotion abnormalities are highly prevalent in various cardiovascular and inflammatory diseases. It remains unknown whether active myocardial inflammation due to cardiac sarcoidosis is associated with coronary vasomotion abnormalities.Case summary A 68-year-old man without a past medical history experienced an out-of-hospital cardiac arrest due to ventricular fibrillation and was successfully resuscitated without neurologic sequelae. Coronary angiography showed normal coronary arteries; however, intracoronary acetylcholine provocation testing demonstrated both epicardial coronary and coronary microvascular spasm. He was diagnosed with isolated cardiac sarcoidosis by fulfilling the diagnostic criteria proposed by the Japanese Circulation Society 2016 diagnostic guidelines, including fatal ventricular arrhythmia, focal left ventricular wall asynergy, increased myocardial fluorodeoxyglucose uptake by positron emission tomography, and late gadolinium enhancement by cardiac magnetic resonance in the heart. He was treated with calcium-channel blocker for coronary artery spasm and prednisolone for cardiac sarcoidosis and underwent implantation of an implantable cardioverter-defibrillator for secondary prevention. Following the treatment, the severity of coronary artery spasm was reduced along with regression of the myocardial inflammation.Discussion Epicardial coronary artery and coronary microvascular spasm can be accompanied by active myocardial inflammation of isolated cardiac sarcoidosis, and the treatment with corticosteroid and calcium-channel blocker may be effective for relieving the severity of coronary artery spasm in association with regression of myocardial inflammation of the disease.
| S-EPMC9157505 | 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