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Brain & Heart Cardiac sarcoidosis with AVB and VA

Table 3. Indications for ICD implantation in patients with CS according to the 2014 HRS expert consensus, the 2017
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ACC/AHA/HRS statements, and the 2022 ESC guidelines 39
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Class of ICD recommendations in patients with CS
recommendation HRS expert consensus 2014 ACC/AHA/HRS statements 2017 ESC guideline 2022
1 Sustained VT, cardiac arrest, or LVEF<35% with expected survival of >1 year despite optimal medical therapy
2a LVEF >35% and evidence of scar on CMR/PET after resolution
of inflammation with expected survival of >1 year
2a Syncope secondary to arrhythmia with LVEF >35%
2a Inducible sustained VA on EP study with LVEF >35% and LVEF 35%–50% and minor LGE on CMR after resolution of
expected survival >1 year inflammation with sustained monomorphic VT on EP study
2a Indication of permanent pacing and LVEF >35%
2b LVEF 36%–49% and/or RVEF <40% despite optimal
medical therapy for heart failure and a period of
immunosuppression
Abbreviations: ACC: American College of Cardiology; AHA: American Heart Association; CMR: Cardiac magnetic resonance imaging; CS: Cardiac
sarcoidosis; EP: Electrophysiology; ESC: European Society of Cardiology; HRS: Heart Rhythm Society; ICD: Implantable cardioverter defibrillator;
PET: Positron emission tomography; LGE: Late gadolinium enhancement; LVEF: Left ventricular ejection fraction; RVEF: Right ventricular ejection
fraction; VA: Ventricular arrhythmia; VT: Ventricular tachycardia.

Table 4. ICD indications in the studies referenced in Figure 1 have evaluated ICD therapy specifically, its significance,
(n=Number of patients) despite the risks of complications and inappropriate
therapy, was highlighted by Nordenswan et al., who found a
Indication for ICD Mohsen et al. Mathijssen et al.
(n=30) 40 (n=105) 44 VA rate of 24.6% (n = 98) (sustained VT or sudden cardiac
Ventricular arrhythmia 16 19 death) over 5 years using the Myocardial Inflammatory
Diseases in Finland Study Group Registry.
41
AVB/Bundle branch block 9 46
Others (reduced LVEF, 5 40 A challenge arises when a pacemaker is indicated and CS
syncope, sudden cardiac death) is suspected but not yet confirmed. Proceeding immediately
Abbreviations: AVB: Atrioventricular block; ICD: Implantable to ICD implantation has the risk of inappropriate shocks
cardioverter defibrillator; LVEF: Left ventricular ejection fraction. but prevents the need for additional procedures, where a
pacemaker is initially placed and later upgraded to an ICD.
Temporary pacing may be used during diagnostic imaging,
40
and clinical suspicion can help guide decision-making. At
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present, there are no established guidelines for such cases;
30 therefore, treatment must be decided by the clinician on a
25 case-by-case basis.
20 Appropriate Therapy
Inappropriate Therapy 3.3. Antiarrhythmic drugs
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10 Antiarrhythmic drugs are commonly used with an ICD
to treat VAs, although their effectiveness has not been
5
extensively studied. A multicenter prospective study on
0
Mohsen (2013) Mathijssen (2022) medical therapy for VAs recommended a stepwise approach:
starting with an ICD alone, followed by the addition of
Figure 1. Comparison of appropriate and inappropriate implantable
cardioverter defibrillator therapy administered over 9- and 8-year an immunosuppressant and an antiarrhythmic drug, and
follow-up periods, respectively (x-axis = Percentage of patients receiving finally using radiofrequency ablation. In this study, 12
therapy). Figure created by author of the 21 patients were managed with medical therapy
alone, and 5 of the 9 patients who needed antiarrhythmics
and programmed electrical stimulation were associated were prescribed amiodarone. However, specific data on
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with an 8.6- and 9-fold increased risk of VAs, respectively, antiarrhythmic use for VAs in CS are limited. Sotalol and
and positive LGE and PET were correlated with a 6.8- and amiodarone are generally recommended because VAs in
3.4-fold increased risk of major adverse cardiac events, patients with CS often stem from re-entry or scar tissue,
respectively. Although no prospective randomized trials whereas beta blockers are suggested for their additional
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Volume 2 Issue 4 (2024) 8 doi: 10.36922/bh.3515