Effect of Corticosteroid Therapy in Patients with Cardiac Sarcoidosis on Frequency of Venous Thromboembolism

Introduction: It is widely known that ventricular tachy-arrhythmias (VTs) are often observed in patients with cardiac sarcoidosis (CS) as one of the presentations of poor prognosis. However, the time-course and influencing factors of VTs after the introduction of corticosteroid therapy in CS patients remain to be elucidated. Methods and Results: We examined the influence of steroid therapy on VTs in 68 consecutive CS patients in Tohoku University Hospital from October 1998 until September 2014 (57±11 years-old, M/F 18/50). CS was diagnosed based on the original guidelines for diagnosis of CS from the Japanese Ministry of Health and Welfare. Corticosteroid therapy was performed in all CS patients. VTs were defined as sustained ventricular tachycardia/fibrillation or appropriate ICD therapy events. During a mean follow-up of 5.5 years, 20 out of 68 patients (29%) experienced VTs after the initiation of corticosteroid therapy, and 14 (70%) of them had VTs in the first 12 months (Figure A). Multivariable analysis showed that the positive gallium scintigraphy was an independent risk factor for VTs (odds ratio, 17.4; 95% CI, 1.03-294, P=0.047). Survival free from VTs events was significantly lower in the Ga-positive group than in the Ga-negative group (P<0.001), where most of the events were developed in the early phase in the Ga-positive group (Figure B). Furthermore, electrical storm (ES) of VTs was noted in 10 patients (14.7%), and most of them (8 out of the 10 patients) had ES in the first 12 months, whereas the recurrence of ES was relatively low (Figure C). Conclusions: VTs or ES in CS patients was frequently noted in the first 12 months after initiation of corticosteroid therapy, possibly representing inflammatory conditions, for which Ga scintigraphy is useful diagnostic strategy and Ga-positive is an independent prognostic factor in CS.

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Effect of Corticosteroid Therapy on Ventricular Arrhythmias in Patients with Cardiac Sarcoidosis

Annals of Noninvasive Electrocardiology ◽

10.1111/j.1542-474x.2011.00418.x ◽

2011 ◽

Vol 16 (2) ◽

pp. 140-147 ◽

Cited By ~ 105

Author(s):

Kenji Yodogawa ◽

Yoshihiko Seino ◽

Toshihiko Ohara ◽

Hideo Takayama ◽

Takao Katoh ◽

...

Keyword(s):

Corticosteroid Therapy ◽

Ventricular Arrhythmias ◽

Cardiac Sarcoidosis

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Recurrences of disease activity in patients with cardiac sarcoidosis under corticosteroid therapy: prevalence, clinical background and prognosis

European Heart Journal ◽

10.1093/ehjci/ehaa946.2135 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

K Kaneta ◽

H Takahama ◽

E Tateishi ◽

K Moriuchi ◽

M Amano ◽

...

Keyword(s):

Disease Activity ◽

Corticosteroid Therapy ◽

Cardiac Sarcoidosis ◽

Univariate Analysis ◽

Prognostic Significance ◽

Laboratory Data ◽

Left Ventricular ◽

Kaplan Meier ◽

Number Of Patients

Abstract Background Corticosteroid therapy (CTx) has been widely accepted as first-line therapy for cardiac sarcoidosis (CS), but there are very limited data regarding recurrence of disease activity of CS. We retrospectively investigated the prevalence, patient characteristics and prognostic significance in patients with recurrences of CS. Methods We identified 102 consecutive patients who were clinically diagnosed CS (admission: 2012 and 2019) and whose disease activity was diminished clinically at least once. Recurrence of CS was defined as detection of increased uptake of 18F-fluoro-2-deoxyglucose or gallium-67 in the follow-up examination. Composite adverse events (events) were defined as all-cause of death or hospitalization for heart failure. Echocardiographic and laboratory data before initiation of CTx were obtained. Results During the follow-up term (median: 4.9 years), the recurrences of CS occurred in 28 patients at 30 months (median) after the initiation of CTx. In patients with recurrence, left ventricular (LV) ejection fraction before initiation of CTx was lower than in those without recurrences (median: 31% vs. 39%, p<0.05). After the detection of CS recurrences, 17 patients were treated with only increases of PSL and remaining 11 patients were treated with adding other immunosuppressive therapies to CTx. The univariate analysis demonstrated that there were no parameters in echocardiographic and laboratory examinations to predict the recurrences of CS. The results of univariate analysis for event occurrences (n=12) are shown in the Table. Additionally, the Kaplan-Meier analysis showed that there were no differences in event free survival rate in the patients with and without CS recurrences (Figure). Conclusion This study showed that the recurrences of disease activity were observed in a substantial number of patients with CS even under the CTx. All patients received intensification of CTx or additional immunosuppressive therapy, and LV systolic function, rather than the recurrence itself, was associated with clinical outcomes in this study. Kaplan-Meier analysis for events Funding Acknowledgement Type of funding source: None

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Long time clinical course of cardiac sarcoidosis with corticosteroid therapy -from Japanese nationwide questionnaire survey-

European Heart Journal ◽

10.1093/ehjci/ehaa946.2109 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

H Kamada ◽

K Ishibashi ◽

K Nakajima ◽

N Ueda ◽

T Kamakura ◽

...

Keyword(s):

Ventricular Arrhythmia ◽

Corticosteroid Therapy ◽

Questionnaire Survey ◽

Clinical Characteristics ◽

Cardiac Involvement ◽

Cardiac Sarcoidosis ◽

Japanese Patients ◽

Left Ventricular ◽

Left Ventricular Ejection

Abstract Background Sarcoidosis is a systemic inflammatory syndrome of unknown etiology and cardiac involvement has been reported to be an important prognostic factor in this disease. An autopsy study has reported that the frequency of this cardiac involvement (cardiac sarcoidosis: CS) varies in the different countries and races and very frequent in Japanese patients. We therefore performed the nationwide questionnaire survey and try to clarify the clinical characteristics and corticosteroid effect in CS, especially focused on arrhythmic events in this disease. Methods Total of 757 Japanese patients from 57 hospitals who diagnosed CS were examined. Patients who unsatisfied the criteria of the Japanese new guidelines, or who underwent cardiac transplantations were excluded, and 420 patients (287 females, median follow-up periods 1864 days [interquartile range: 845–3159 days]) were analyzed. The clinical outcome and corticosteroid effect were evaluated. Results Clinical characteristics at diagnosis was as follows: female dominant (68%), mean age of 60±13 years old, mean left ventricular ejection fraction was 49±16%. Arrhythmic events were very frequently observed as an initial cardiac manifestation in 263 patients (62%) of CS, of which atrioventricular block (AVB) in 174 (41%), ventricular tachycardia (VT) in 73 (17%) and AVB with VT in 17 (4%) (Figure 1A). Pacemaker was implanted in 166 patients (40%) and defibrillators was 137 patients (33%). Corticosteroid was prescribed in 144 (83%) of 174 patients with AVB and in 62 (85%) of 73 patients with VT. Initial dose was mean 47.9 mg and maintenance dose of mean 7.3 mg. Corticosteroid improved VT as good as AVB (27% vs. 29%). However, corticosteroid sometimes worsened VT events compared with AVB (10% vs. 2%) (Figure 1B). During the course of follow-up, 32 patients were needed to increase corticosteroid in 23 of AVB and 10 of VT cases. However, there were no difference in mortality between the groups, whether or not to increase corticosteroid. All survival rate was 92% (5-year mortality), 83% (10-year mortality) and free from all cause death and defibrillator charge was 81% (5 year), 71% (10 year). Conclusion Fatal arrhythmia is commonly observed in CS as a primary symptom. Corticosteroid sometimes worsen ventricular arrhythmia and appropriate defibrillator discharge was common. Thus, careful attention for activating ventricular arrhythmia would be needed during the follow-up period even after corticosteroid therapy. Funding Acknowledgement Type of funding source: None

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Conduction disorders as the first hallmark of isolated cardiac sarcoidosis in a highly active individual: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytab416 ◽

2021 ◽

Author(s):

Silvia Muccioli ◽

Stefano Albani ◽

Barbara Mabritto ◽

Giuseppe Musumeci

Keyword(s):

Corticosteroid Therapy ◽

Cardiac Sarcoidosis ◽

Multimodality Imaging ◽

Electrophysiological Study ◽

Cardiac Conduction ◽

Moderate Physical Activity ◽

Middle Aged ◽

High Intensity Training ◽

Highly Active ◽

Active Individual

Abstract Background Cardiac sarcoidosis (CS) is an inflammatory disease with various clinical presentations depending on the extension of cardiac involvement. The disease is often clinically silent, therefore diagnosis is challenging. Case summary We discuss the case of a middle-aged highly active individual presenting with an occasional finding of low heart rate during self-monitoring. The electrocardiogram shows a Mobitz 2 heart block; thanks to multimodality imaging CS was diagnosed and corticosteroid therapy improved cardiac conduction. Discussion To our knowledge this is one of the first documented cases of occasional, early finding of CS in a middle-aged highly active individual who presented with cardiac conduction involvement. Despite the very early diagnosis, multimodality imaging suggested an advanced disease with no edema detection at the CMR. Nevertheless, prompt corticosteroid therapy was able to improve clinical conduction. Although non-sustained ventricular arrhythmias were detected, electrophysiological study allowed to discharge the patient safely without implantable cardioverter defibrillator implantation. Light-to moderate physical activity was allowed at mid-term follow up. A multidisciplinary evaluation should be considered to resume a high intensity training.

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Abstract 2126: Therapeutic Evaluation of Corticosteroid Therapy for Ventricular Tachycardia Associated with Cardiac Sarcoidosis -Application of High Resolution Electrocardiography-

Circulation ◽

10.1161/circ.118.suppl_18.s_671-a ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Kenji Yodogawa ◽

Yoshihiko Seino ◽

Norihiko Ono ◽

Chikao Ibuki ◽

Toshihiko Ohara ◽

...

Keyword(s):

Ventricular Tachycardia ◽

Corticosteroid Therapy ◽

Cardiac Sarcoidosis ◽

Holter Monitoring ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Qrs Complex ◽

Ventricular Ejection Fraction ◽

Arrhythmogenic Substrate ◽

Therapeutic Evaluation

Background The outcome of cardiac sarcoidosis (CS) is sometimes fatal. Ventricular tachycardia (VT) is one of the common causes of sudden death in CS patients. In patients with definite diagnosis of CS, corticosteroid therapy should be the absolute indication. However, little is known about the effectiveness for VT. Further, there is no established method for therapeutic evaluation in CS. Thus, we investigated whether or not VT is suppressed following corticosteroid therapy and the utility of Signal Averaged Electrocardiography (SAECG) for therapeutic assessment in CS patients with VT. Methods Fifteen histological proved CS patients presenting with sustained or non-sustained VT were investigated. All of these patients were treated with predonisolone 30mg to 10mg/day, and assessed before and after corticosteroid therapy. All patients underwent SAECG in which the filtered QRS duration (f-QRS), the root mean square voltage of the terminal 40 ms (RMS 40 ) in the filtered QRS complex and the duration of low-amplitude signals < 40μV (LAS 40 ) in the terminal filtered QRS complex were measured. The presence of VT was assessed by Holter monitoring. Results VT was suppressed in 6 patients {VT (−) group} and the remaining 9 patients were not {VT (+) group}. Accumulation of gallium-67 was detected more frequently in VT (−) group than in VT (+) group (66.7% vs. 11.1%, p<0.05). Left ventricular ejection fraction (LVEF) was significantly higher in VT (−) group than that of VT (−) group (54.1+/−20.1 vs 32.8+/−11.7 p<0.05). In VT (−) group, f-QRS and LAS 40 were significantly decreased and RMS 40 was significantly increased compared with those before corticosteroid therapy (f-QRS: 136.3+/−30.6msec vs 116.8+/−25.4msec, p<0.05 LAS 40 : 68.2+/−24.0msec vs 47.8+/−22.9msec, p<0.05 RMS 40 : 7.2+/−3.3 msec vs 13.3+/−7.6msec, p<0.05). However, SAECG parameters did not change significantly in VT (+) group. Conclusions In the early and viable stage of the disease, corticosteroid therapy was effective for VT in CS. Reversible conduction abnormality detected by SAECG might reflect reversible arrhythmogenic substrate for the occurrence of VT. SAECG is useful for therapeutic evaluation of CS patients with VT.

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