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Sudden Cardiac Death and Life-Threatening Arrhythmias in Cardiac Sarcoidosis
Quick Takes
- Almost all patients with clinically manifest cardiac sarcoidosis will benefit from an implantable cardioverter-defibrillator (ICD).
- A positive myocardial biopsy identifies patients at highest risk of a life-threatening ventricular arrhythmia.
- Serial risk assessment is appropriate in the minority of patients with cardiac sarcoidosis who do not have a Class I or IIa indication for an ICD.
Study Questions:
How effective are the current implantable cardioverter-defibrillator (ICD) guidelines for identifying patients with cardiac sarcoidosis (CS) who are at risk of sudden cardiac death (SCD) or sustained ventricular tachycardia (SVT)?
Methods:
The subjects of this study were 398 patients with CS who were entered into a Finnish registry in 1988–2017. The diagnosis of CS was considered definite in 193 patients who had a positive myocardial biopsy and probable in 205 patients based on the Heart Rhythm Society (HRS) diagnostic criteria. The primary outcome was SCD (actual or aborted), or SVT during follow-up in patients with and without an ICD.
Results:
Based on HRS guidelines, 85% of patients had a Class I or IIa indication for an ICD upon entry into the registry. A total of 294 patients (74%) received an ICD. During a median of 4.8 years of follow-up, the annual incidence of SCD was 10.7% in patients with an ICD and 4.8% in patients without an ICD. By multivariate analysis, a positive myocardial biopsy was an independent predictor of SCD, but the presence of a Class I or IIa indication was not. Among the patients without a Class I or IIa indication for an ICD upon entry into the registry, the 5-year incidence of SCD, SVT, or the emergence of a Class I/IIa ICD indication was 53%.
Conclusions:
Patients with CS who do not present with a Class I/IIa indication for an ICD have approximately a 5% annual risk of SCD. All patients with clinically manifest CS may be appropriate candidates for an ICD.
Perspective:
The findings of this study make it clear that all patients with CS and a positive myocardial biopsy should receive an ICD. In addition, an ICD should be strongly considered, even if a Class I/IIa ICD indication is not present, in patients with probable CS. The HRS criteria for probable CS consist of a positive extracardiac biopsy plus one or more of the following: corticosteroid or immunosuppressive therapy responsive cardiomyopathy or heart block; high-degree heart block, a left ventricular ejection fraction <50%, patchy uptake on fluorodeoxyglucose positron emission tomography, late gadolinium enhancement on cardiac magnetic resonance imaging, or a positive cardiac gallium scan.
Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Computed Tomography, Magnetic Resonance Imaging, Nuclear Imaging
Keywords: Arrhythmias, Cardiac, Biopsy, Cardiomyopathies, Death, Sudden, Cardiac, Defibrillators, Implantable, Diagnostic Imaging, Gadolinium, Heart Block, Heart Failure, Immunosuppression, Magnetic Resonance Imaging, Positron-Emission Tomography, Risk, Sarcoidosis, Tachycardia, Ventricular, Ventricular Function, Left
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