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Brain & Heart Cardiac sarcoidosis with AVB and VA

Table 2. (Continued)
Author Place of Type of study No. of No. of patients Endpoints/outcomes Key findings
study patients with with VAs
AVB
Nordenswan Finland Case–control study 143 20 patients with Sudden cardiac death at The risk of VA was high in patients
et al. 2018 53 AVB presentation with VA initially presenting with AVB
and remained higher if it was
concomitant with VA or severe LVSD
at the time of diagnosis
Takaya et al. Japan Retrospective 22 31 (including Endpoint was major Patients with AVB and VA have a
2015 54 single-center cohort presentation adverse cardiac outcomes: similar mortality, although those with
with heart cardiac death, VA, VAs may have a higher incidence of
failure) hospitalization for heart major adverse cardiac events
failure
Abbreviations: 18F-FDG PET: 18 Fluorodeoxyglucose positron emission tomography; AVB: Atrioventricular block; CS: Cardiac sarcoidosis;
ICD: Implantable cardioverter defibrillator; LGE: Late gadolinium enhancement; LVEF: Left ventricular ejection fraction; LVSD: Left ventricular
systolic dysfunction; RV: Right ventricle; VA: Ventricular arrhythmia; VT: Ventricular tachycardia.

Steroid-sparing agents are commonly used as second- Owing partially to concurrent immunosuppression,
line treatments or adjuncts to corticosteroids. Among patients with CS who undergo ICD implantation face a high
these, methotrexate is the most prominent and extensively risk of complications, including infections or lead fractures
studied. Although some reduction in myocardial and dislodgement, affecting up to 15% of these patients.
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inflammation has been observed with methotrexate In addition, an analysis by Kron et al. suggested that
through 18F-FDG PET, specific outcomes related to VAs patients with isolated CS and ICDs had poorer outcomes
and AVB have not been widely reported. Infliximab than those with sarcoidosis involving other systems. This
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use has been explored for cases refractory to second-line patient group had very high rates of VAs and appropriate
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agents. A recent case series reported no major arrhythmic shocks (69%) over 4 years. However, selection bias may
events (VA or AVB) in six patients over a mean follow-up influence these findings, as isolated CS is less likely to
period of 21.2 ± 15.5 months, and five of them had be diagnosed or could be mistaken for other conditions,
presented with VA or AVB at baseline. In addition, MRI potentially leading to the diagnosis of only more severe
during follow-up showed a reduction in FDG PET activity cases. Factors associated with worse outcomes in this group
in all patients, indicating significant improvement in include the presence of LGE on CMR, worsened New York
disease activity. Larger, multicenter studies are warranted Heart Association functional classification of heart failure,
36
to further evaluate the efficacy of infliximab. reduced LVEF, and comorbidities such as chronic kidney
and lung diseases. 43
3.2. Implantable cardioverter defibrillator (ICD)
Recent advancements in device leads and the increased
ICDs are considered as a Class 1 indication for VAs and a use of home monitoring for device interrogation have led
Class 2a indication for AVB, even if the AVB temporarily to lower rates of inappropriate shocks than those previously
reverses. This recommendation is based on the high risk reported. For example, Mathijssen et al. recorded an
of future VAs, as outlined in Table 3. 15,37,38 Pacemaker inappropriate shock rate of 2.9%, as shown in Figure 1
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indications, as specified by Glikson et al. in the 2021 ESC and Table 4. This study found that ICDs used for secondary
guidelines, primarily include complete heart block, Mobitz prevention (class 1 indication) delivered appropriate
II heart block, Mobitz I with syncope or infranodal block, therapy more frequently than those used for primary
and symptomatic sinus node disease. ICD implantation prevention (class 2A indication) over a median follow-up
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has been shown to significantly reduce mortality. For period of 2.8 years (82% vs. 19%), which aligns with
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example, Nery et al. reported that seven patients with CS international guidelines. 15,37,38 Predictors of appropriate
underwent ICD implantation after initially presenting therapy included prior VAs and RV LGE on CMR,
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with AVB, with two of them subsequently receiving shocks differing from a previous meta-analysis that identified
for recurrent VT over an average follow-up period of 21 younger age, male sex, lower LVEF, complete heart block,
± 9 months.11 Another retrospective study revealed that and ventricular pacing as significant predictors. LGE in
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although 36.7% of patients received appropriate shocks, the RV has been linked to higher rates of sustained VT
30% received inappropriate shocks, and no significant than that in other regions. A meta-analysis assessing the
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predictors of such tachyarrhythmias were identified. risk of sudden cardiac death reported that positive LGE
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Volume 2 Issue 4 (2024) 7 doi: 10.36922/bh.3515