Implantable cardiac devices in cardiac sarcoidosis: it is about preventing the unforeseeable

We read with interest the recent publication by Park et al.1 In their case report, the authors present a case of cardiac sarcoidosis (CS) manifesting with a high-degree atrioventricular block (AVB). Despite the unusual young age of the patient (CS typically occurs in patients between 30 and 60 years of age),2,3 findings from imaging and histology, convincingly support the diagnosis. Corticosteroid treatment resulted in the normalization of atrioventricular conduction, persuading managing physicians that a permanent device was not needed. Among CS patients presenting with AVB, atrioventricular conduction may recover in perhaps 50% of patients under immunosuppressive treatment.4 We agree that the decision to implant a device in a young patient should always be carefully considered. However, there are two additional factors that need to be discussed. First is the substantial risk for malignant arrhythmias. In a study of 22 Japanese CS patients with a high-grade AVB, over a median follow-up of 34 months, 2 patients suffered aborted sudden cardiac death (SCD) and 9 had sustained ventricular tachycardia (VT).5 Nordenswan et al. reported on the arrhythmic outcomes of 143 CS patients who presented with Mobitz II or 3rd degree AVB. Over a median follow-up period of 4.1 years, 23 (16.1%) patients suffered either fatal (13) or aborted (10) SCD.3 An additional 21 patients had sustained VT and the combined end-point of SCD/VT occurred in 44/143 (30.8%). Importantly, the annual rate of the combined end-point in the patients like the case presented (i.e. with normal left ventricular ejection fraction) was 5%.3 These and other data have led all major guidelines to recommend implantable cardioverter defibrillator (ICD) in patients like the one described in the present case report.6 The other issue is the high likelihood, unpredictability, and potential sequelae of disease relapse. In this regard, we would be interested to know the authors’ plan for the duration of therapy. We recently showed that in similar patients to the one presented, 12/19 (63.1%) relapsed on follow-up positron emission tomography scan after prednisone was stopped (after 1 year of therapy).2 The mechanisms behind relapsing CS are poorly understood, and currently, no tools exist to predict which patients will be affected. In conclusion, we would have strongly recommended ICD implantation to this patient. Of course, we also support the shared decision-making process and understand that device therapy has potential downsides. Hence, it follows that after sharing all the pros and cons, then a patient may opt to not have a device implanted. Funding: A.D.B. is supported by restricted grants provided by the Caroline Musæus Aarsvolds Fund, Tom Wilhelmsen's Foundation, Gidske and Peter Jacob Sørensens Fund, and by Vestfold Hospital Trust.

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