Implantable Cardioverter Defibrillator Therapy in Patients with Cardiac Sarcoidosis

Abstract

ICD Shocks in Cardiac Sarcoidosis. Background: An implantable cardioverter defibrillator (ICD)is indicated for some patients with cardiac sarcoidosis (CS) for prevention of sudden death. However, there are little data regarding the event rates of ICD therapies in these patients. We sought to identify the incidence and characteristics of ICD therapies in this patient population.Methods: We performed a cohort study of patients with ICDs at 3 institutions. Cases were those patients with CS and an ICD implanted for primary or secondary prevention of sudden death. Additionally, we included a comparison with historical controls of ICD therapy rates reported in clinical trials evaluating the ICD for primary and secondary prevention of sudden death.Results ]).Conclusions: In our cohort of patients with CS and ICDs, almost one-third receive appropriate therapies. This may be due to a myocardial inflammatory process leading to increased triggered activity and subsequent scarring leading to reentrant tachyarrhythmias. Adjusted predictors of ICD therapies in this population include left or right ventricular dysfunction. (J Cardiovasc Electrophysiol, Vol. 23, pp. 925-929, September 2012) cardiac sarcoid, cardiomyopathy, heart failure, implantable cardioverter defibrillator, ventricular tachycardia

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

“…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.…”

“…Our findings support these 2 data sets but also add to the knowledge by identifying impaired RVEF and basal septal thinning as CMRI variables portending poorer outcome. The predictive value of RV function had also been suggested in an earlier study by Schuller et al24 revealing that RV dysfunction, shown by echo, nuclear imaging, or MRI, predicted life‐threatening arrhythmias in CS patients with an ICD. In addition, the data of Crawford et al11 and Blankstein et al25 suggest that life‐threatening arrhythmic events in CS may be related to inflammatory involvement of the RV wall.…”

“…Most of the comparable previous work11, 12, 19, 24, 25, 27 relied mainly on the Japanese diagnostic criteria,22, 23 which suffer from problems with sensitivity and specificity3, 4, 8, 10, 29 and are no longer endorsed by experts in the field 3. The generic limitations of retrospective studies apply to our work, as they do to all other outcome data in CS because of the lack of prospective research.…”

“…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.…”

Right ventricular involvement and the extent of left ventricular enhancement with magnetic resonance predict adverse outcome in pulmonary sarcoidosis

“…DECMR has become the gold standard test for detecting and quantifying focal myocardial scar, a predictor of high degree atrio‐ventricular block (AVB), heart failure [congestive cardiac failure (CCF)], ventricular tachycardia (VT), and sudden cardiac death (SCD) in CS 2, 3, 4, 5, 6, 7, 8, 10, 11, 12, 13, 14, 15. ( Table 1A and 1B) Systolic right ventricular (RV) impairment and multi‐focal RV late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) and active RV granulomatous inflammation on PET have been associated with adverse outcomes in retrospective studies 2, 7, 11, 12, 16, 17, 18. We previously demonstrated the diagnostic accuracy of DECMR for CS and reported on RV involvement in pulmonary sarcoidosis utilizing CMR 19, 20.…”