Efficacy and safety of implantable cardiac defibrillators for treatment of ventricular arrhythmias in patients with cardiac sarcoidosis

Kron, Jordana; Sauer, William H.; Schuller, Joseph L.; Bogun, Frank; Crawford, Thomas; Sarsam, Sinan; Rosenfeld, Lynda E.; Mitiku, Teferi; Cooper, Joshua M.; Mehta, Davendra; Greenspon, Arnold J.; Ortman, Matthew; Delurgio, David B.; Valadri, Ravinder; Narasimhan, Calambur; Swapna, Nalla; Singh, Jagmeet P.; Danik, Stephan B.; Markowitz, Steven M.; Almquist, Adrian K.; Krahn, Andrew D.; Wolfe, Luke; Feinstein, Shawn; Ellenbogen, Kenneth A.

doi:10.1093/europace/eus316

Cited by 157 publications

(108 citation statements)

References 17 publications

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“…We think that the late observations on the predictive value of myocardial LGE, summarized in Table 5, call for a reevaluation of the recommendation at this point. Importantly, LGE predicted cardiac events despite normal or mildly reduced LV function,11 and in the study by Ise et al12 and in our study, it even outperformed LVEF, the key prognostic factor in CS reported previously 1, 2, 8, 24, 29. It is worth discussing whether some definition of myocardial LGE, for example, extent ≥22% of LV mass or involvement of the RV wall or involvement of ≥9 of 29 combined LV and RV segments (Table 5), should be added as a new or strengthening indication for an ICD in patients with CS and normal or mildly reduced LV function.…”

Section: Discussionsupporting

confidence: 73%

“…Finally, ICD “may be considered” (class 2b indication) in patients with LVEF between 36% and 49% and/or RVEF <40%. The last recommendation was based mainly on 3 cohorts of CS patients with an ICD showing that ICD therapies were common, even in patients with mildly reduced LVEF 2, 24, 28. The value of myocardial LGE as an indication for an ICD in patients with normal LVEF was considered but dismissed by the experts 3.…”

Section: Discussionmentioning

confidence: 99%

“…Fatal arrhythmias, instead, explain 90% of cardiac mortality, and aborted arrhythmic deaths are as common as all cardiac fatalities together 1. Implantable cardioverter‐defibrillator (ICD) therapy is able to prevent sudden death in CS,2 but its complications cannot be ignored, and today's experts advocate implantations only in patients at high risk of fatal arrhythmias 3. Individual risk assessment is problematic, however, because there are no large outcome studies in CS.…”

Section: Introductionmentioning

confidence: 99%

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Magnetic Resonance Imaging as a Predictor of Survival Free of Life‐Threatening Arrhythmias and Transplantation in Cardiac Sarcoidosis

Ekström

Lehtonen

Hänninen

et al. 2016

JAHA

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BackgroundCardiac 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 ResultsOur 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.ConclusionsFindings 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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Section: Discussionsupporting

confidence: 73%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Magnetic Resonance Imaging as a Predictor of Survival Free of Life‐Threatening Arrhythmias and Transplantation in Cardiac Sarcoidosis

Ekström

Lehtonen

Hänninen

et al. 2016

JAHA

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“…This is possibly due to the mixture of scar and active inflammation in many patients. As an example, CS patient cohorts seem to have higher rates of ICD therapies than other patients (42,43). The 2014 Heart Rhythm Society consensus document ( Fig.…”

Section: Ventricular Arrhythmiasmentioning

confidence: 99%

Clinical Management of Cardiac Sarcoidosis

Birnie

Nery

2017

Annals of Nuclear Cardiology

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Approximately 5% of patients with sarcoidosis will have cardiac involvement clinically manifest as one or more of ventricular arrhythmias, conduction abnormalities and heart failure. Another 20% to 25% have clinically silent disease (asymptomatic cardiac involvement). There is a growing realisation that CS can be the first manifestation of sarcoidosis in any organ. In particular physicians should consider CS in patients with VT of unknown etiology and in patients aged <60 presenting with idiopathic advanced conduction system disease.Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest CS despite modest data. Positron Emission Tomography (FDG-PET) imaging is often used to detect active disease and guide immunosuppression. The extent of left ventricular dysfunction seems to be the most important predictor of prognosis. Also the extent of LGE on CMR is emerging as an important prognostic factor. Patients with clinically manifest disease often need device therapy, usually with implantable cardioverter defibrillators. There are still much to be learned as regarding best practices in managing CS patients and multicenter research efforts are underway.

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“…It has been reported that VTs are associated with reduced LVEF or a history of VTs in CS patients. 5,31,32 It was reported that delayed enhancement in the right ventricle on cardiac MRI is related to VTs in CS patients with preserved LV function and no history of VTs. 31 These findings may indicate the existence of the reentrant mechanisms for VTs.…”

Section: Positive Gallium Scintigraphy As a Factor Correlating With Vtsmentioning

confidence: 99%

Time Course and Factors Correlating With Ventricular Tachyarrhythmias After Introduction of Steroid Therapy in Cardiac Sarcoidosis

Segawa

Fukuda

Nakano

et al. 2016

Circ: Arrhythmia and Electrophysiology

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Background— The time course and factors correlating with ventricular tachyarrhythmias (VTs) after introduction of corticosteroid therapy in patients with cardiac sarcoidosis remain to be elucidated. Methods and Results— We examined 68 consecutive patients with cardiac sarcoidosis in the Tohoku University Hospital from October 1998 to September 2014 (age: 57±11 years old; male:female 18:50) and evaluated VTs after initiation of steroid therapy. VTs were defined as documented ventricular tachycardia or ventricular fibrillation lasting for more than 30 seconds or resulting in cardiovascular collapse, or appropriate implantable cardioverter defibrillator therapy. During a mean follow-up of 5.5 years, 20 out of 68 patients (29%) experienced VTs after initiation of corticosteroid therapy, especially in the first 12 months in 14 patients (70%). A multivariable analysis revealed that positive gallium scintigraphy had a significant correlation with VTs (hazard ratio, 11.33; 95% confidence interval, 3.22–39.92; P <0.001), in addition to reduced left ventricular ejection fraction (hazard ratio, 0.94; 95% confidence interval, 0.90–0.97; P =0.001). Furthermore, electrical storm was noted in 10 patients (14.7%), 8 within the first 12 months of treatment, whereas the recurrence of electric storm was relatively less. Conclusions— These results indicate that VTs and electric storm frequently occur in the first 12 months after initiation of corticosteroid therapy, presumably because of inflammatory conditions, and that the positive gallium scintigraphy is a significant and independent predictor of VTs. The present findings may be useful to further improve the management of VTs in patients with cardiac sarcoidosis.

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Efficacy and safety of implantable cardiac defibrillators for treatment of ventricular arrhythmias in patients with cardiac sarcoidosis

Abstract: Patients with CS and ICDs are at high risk for ventricular arrhythmias. This population also has high rates of inappropriate shocks and device complications.

Cited by 157 publications

References 17 publications

Magnetic Resonance Imaging as a Predictor of Survival Free of Life‐Threatening Arrhythmias and Transplantation in Cardiac Sarcoidosis

Magnetic Resonance Imaging as a Predictor of Survival Free of Life‐Threatening Arrhythmias and Transplantation in Cardiac Sarcoidosis

Clinical Management of Cardiac Sarcoidosis

Time Course and Factors Correlating With Ventricular Tachyarrhythmias After Introduction of Steroid Therapy in Cardiac Sarcoidosis

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