Management Of Cardiac Sarcoidosis Research Articles

Roles of <sup>18</sup>F-FDG PET in Diagnosis and Management of Cardiac Sarcoidosis—from the Continuing Medical Education Session at the 63<sup>rd</sup> SNMMI Meeting, June 2016

Roles of <sup>18</sup>F-FDG PET in Diagnosis and Management of Cardiac Sarcoidosis—from the Continuing Medical Education Session at the 63<sup>rd</sup> SNMMI Meeting, June 2016

Cardiac Arrhythmias as Initial Presentation of Sarcoidosis

SESSION TITLE: Student/Resident Case Report Poster - Cardiovascular Disease I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Cardiac sarcoidosis is an infiltrative disease which is usually clinically silent but occasionally may be the first manifestation of sarcoidosis even in the absence of overt systemic involvement. It frequently presents with structural and conduction abnormalities including supraventricular and ventricular arrhythmias, myocardial damage simulating infarction, heart failure, cardiomyopathy, valvular heart disease, and sudden death. CASE PRESENTATION: A 33 year old African American female presented to emergency room with episodes of palpations. Her exam and laboratory work up were unremarkable. Electrocardiogram showed sinus arrhythmia with pre-ventricular contractions and she was started on Metoprolol. She was having non-sustained ventricular tachycardia (NSVT) which subsequently became sustained requiring Lidocaine with conversion to sinus tachycardia. Echocardiography was normal. However she continued to have NSVT which raised a concern for an infiltrative process. She had cardiovascular magnetic resonance imaging (CMRI) which showed a large area of delayed mid myocardial enhancement at the inferolateral, basal, mid portion and apical wall with 25% viability and hypokinesis. A high resolution computed tomography of her chest was unrevealing. Endomyocardial biopsy (EMB) was considered as she had no other obvious manifestation of sarcoidosis but was not done as her lesions were patchy. She eventually got an Implantable Cardioverter-Defibrillator and was discharged on Metoprolol. DISCUSSION: The diagnosis and management of cardiac sarcoidosis is a major challenge especially in the absence of systemic manifestations. Findings on electrocardiogram and echocardiogram are nonspecific; CMRI and even positron emission tomography (PET) features may be suggestive but are not diagnostic. EMB which demonstrates noncaseating epithelioid granulomas lacks sensitivity as it may miss the lesion and is usually reserved for patients without histologic confirmation from another source. The ability to diagnose this promptly, predict progression and prognosis, and intervene before a fatal cardiac event, deserves further investigation. The use of steroids lack confirmatory randomized clinical trials. A low threshold for invasive procedures is suggested due to the risk of sudden death. CONCLUSIONS: In young patients presenting with cardiac arrhythmias, sarcoidosis should be considered even in the absence of systemic involvement as this might be a herald sign for an opportunity to intervene and prevent impending sudden cardiac death. Reference #1: Schulte W, Kirsten D, Drent M, Costabel U.Cardiac involvement in sarcoidosis. Eur Respir Mon. 2005;32:130 Reference #2: Patel MR, Cawley PJ, Heitner JF, Klem I, Parker MA, Jaroudi WA, et al. Detection of myocardial damage in patients with sarcoidosis. Circulation. 2009;120(20):1969. Reference #3: Nery PB, Leung E, Birnie DH. Arrhythmias in cardiac sarcoidosis: diagnosis and treatment. Curr Opin Cardiol. 2012;27(2):181. DISCLOSURE: The following authors have nothing to disclose: Chukwunweike Nwosu, Deepika Nandiraju, Danila Deliana, Joseph Neri No Product/Research Disclosure Information

Combination therapy with corticosteroid and mycophenolate mofetil in a case of refractory cardiac sarcoidosis

Management of cardiac sarcoidosis (CS) can be challenging. The first-line therapy for this condition is corticosteroids, but other immunosuppressive agents are sometimes co-administered to reduce the dosage of corticosteroid and to thereby avoid steroid-induced adverse effects or to increase its therapeutic efficacy. Mycophenolate mofetil (MMF) is a prodrug of mycophenolic acid, an inhibitor of inosine monophosphate dehydrogenase that acts more selectively on T and B lymphocytes when compared with azathioprine. A 40-year-old man was diagnosed with CS after presenting with ventricular fibrillation. His left ventricular ejection fraction was severely reduced (30%), and cardiac positron emission tomography (PET) showed abnormal uptake of 18F-fluorodeoxyglucose. A cardioverter-defibrillator was implanted and prednisolone (30mg/day) was administered. He was re-admitted with recurrent sustained ventricular tachycardia and a positive PET finding despite a 5-month course of prednisolone, and MMF (1000mg/day) was administered. Six months later, he had not required re-hospitalization for heart failure or arrhythmia. We conclude that combination therapy with MMF and corticosteroids is useful for refractory CS.<Learning objective: Management of cardiac sarcoidosis (CS) can be challenging. Although some immunosuppressive agents are co-administered to reduce the dosage of corticosteroids or to intensify the effect of corticosteroids, the optimal combination regimen has not yet been established. This case report shows that combination therapy with corticosteroid and mycophenolate mofetil was useful for CS that was refractory to corticosteroid monotherapy.>

Arrhythmias in cardiac sarcoidosis

Sarcoidosis is a granulomatous disease of unclear cause and variable presentation. Cardiac involvement can result in life-threatening conditions including heart block, ventricular tachycardia, sudden cardiac death, and heart failure. There is no consensus on the diagnosis and management of cardiac sarcoidosis and a practical update is needed to provide clinicians with guidance. Three recent studies have described cardiac manifestations as the first presentation of sarcoidosis. In one study, cardiac sarcoidosis was found to be the underlying cause in 19% of adults aged less than 55 years presenting with new onset unexplained atrioventricular block. Also, there are increasing reports of patients with isolated cardiac sarcoidosis (i.e., without sarcoid in other organs). Finally, advances in imaging have enhanced our ability to detect myocardial involvement and perhaps follow response to treatment. Cardiac sarcoidosis should be considered in patients aged less than 55 years presenting with unexplained atrioventricular block and in patients with idiopathic cardiomyopathy and sustained ventricular tachycardia. Much remains to be learned about the condition, including the role of steroids and devices in treatment, and the place of advanced imaging in following the response to treatment. Collaborative multicenter studies are required to answer these important clinical questions.

Management of Cardiac Sarcoidosis in the United States: A Delphi Study

No formal guidelines exist to guide physicians caring for patients with sarcoidosis in their screening for management of patients with cardiac sarcoidosis. We conducted a modified Delphi study to investigate if a consensus could be reached on the best approaches for screening for and management of cardiac sarcoidosis.A modified Delphi study design with two rounds of questionnaires was used to investigate if a consensus existed among sarcoid experts in the United States on the best management approaches for cardiac sarcoidosis. Experts were identified based on their national reputation as sarcoid experts and by being actively involved in sarcoidosis clinics at their institutions.Overall agreement was low to moderate. Agreement was reached on the role of history, physical examination, and 12-lead ECG in screening, echocardiogram, Holter monitor, myocardial fluorodeoxyglucose PET scan, and cardiac MRI in workup, and steroids in treatment. Agreement was not reached on the role of signal-averaged ECG in screening, optimum dose of prednisone, use of steroid-sparing agents, and duration of treatment. Several comments underscore the diverse approaches and uncertainty that exist in managing cardiac sarcoidosis.Our study highlights the dilemma that sarcoid experts face in their approach to cardiac sarcoidosis. It also highlights the lack of agreement among sarcoid experts on key aspects of diagnosis and management and stresses the importance of collaborative efforts to investigate the best strategies for screening for and management of cardiac sarcoidosis.

Heterogeneous Myocardial FDG Uptake and the Disease Activity in Cardiac Sarcoidosis

This study evaluated the usefulness of fasting (18)F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) in the diagnosis and management of cardiac sarcoidosis (CS) and compared it with FDG uptake in dilated cardiomyopathy (DCM). Cardiac sarcoidosis may clinically present as DCM but is amenable to systemic corticosteroid therapy if disease activity is high. Although alterations of FDG uptake have been reported in CS, limited information is available on the quantitative estimates of FDG uptake. Fasting FDG-PET was performed in 24 systemic sarcoidosis patients and was compared with 8 age-matched DCM patients. FDG-PET was also performed in 15 age-matched healthy control subjects. Twelve of the 24 sarcoidosis patients had cardiac involvement based on criteria established by the Japanese Ministry of Health and Welfare; the remaining 12 of 24 patients revealed no evidence of cardiac involvement. The myocardial FDG uptake was quantified by measuring the standardized uptake value in 17 myocardial segments in each subject. Coefficient of variation (COV), which equals the standard deviation of uptake divided by the average uptake of 17 segments, was calculated as an index of heterogeneity in the heart. The FDG uptake was distinctly heterogeneous in CS patients. The COV value was significantly greater in CS patients (0.25 ± 0.05) than control subjects (0.14 ± 0.03, p < 0.01), sarcoidosis patients without cardiac involvement (0.14 ± 0.03, p < 0.01), or DCM patients (0.15 ± 0.02, p < 0.01). The COV value in DCM patients was similar to control subjects or sarcoidosis patients without cardiac involvement. The cutoff COV value for the diagnosis of CS was 0.18 (sensitivity: 100%; specificity: 97%). After corticosteroid therapy in CS patients, the COV value was decreased to 0.14 ± 0.06 (p < 0.05) and became essentially similar to the other groups. Heterogeneous myocardial FDG uptake may be a useful diagnostic marker of disease activity for CS.

Resting thallium-201 myocardial SPECT in the management of cardiac sarcoidosis with steroid therapy

Resting thallium-201 myocardial SPECT in the management of cardiac sarcoidosis with steroid therapy