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Risk Stratification of Patients With Cardiac Sarcoidosis
Quick Takes
- The incidence of adverse events, particularly fatal arrhythmic events, in patients with cardiac sarcoidosis is high.
- Among patients with cardiac sarcoidosis, patients with low LVEF, high BNP level, ventricular tachycardia or fibrillation history, and the need for ventricular tachycardia ablation are markers of high risk.
Study Questions:
What are the prognosis and prognostic factors of patients with cardiac sarcoidosis?
Methods:
The authors conducted a retrospective analysis of patients in a multicenter registry, ILLUMINATE-CS, in Japan. Patients fulfilled 2016 Japanese Circulation Society or 2014 Heart Rhythm Society diagnostic criteria. The primary endpoint was a composite of all-cause death, hospitalization for heart failure (HF), and documented fatal ventricular arrhythmia events.
Results:
There were 512 patients. They experienced 148 combined events (56 HF hospitalizations, 99 documented fatal ventricular arrhythmia events, and 49 all-cause deaths) during a median follow-up of 1,042 days. The 10-year estimated event rates for the primary endpoint, all-cause death, HF hospitalizations, and fatal ventricular arrhythmia events were 48, 18, 21, and 32%, respectively. On multivariable Cox regression, a history of ventricular tachycardia or fibrillation (hazard ratio [HR], 2.53; log-transformed B-type natriuretic peptide (BNP) levels (HR, 1.28); left ventricular ejection fraction (LVEF) (HR, 0.94 per 5% increase); and post-diagnosis radiofrequency ablation for ventricular tachycardia (HR, 2.65) independently predicted the primary endpoint.
Conclusions:
The authors concluded that in cardiac sarcoidosis, adverse events are mainly due to fatal ventricular arrhythmia events. Patients with low LVEF, high BNP levels, ventricular tachycardia/fibrillation history, and those requiring ablation to treat ventricular tachycardia are at highest risk.
Perspective:
Historically, cardiac involvement has been reported to be manifest in only 5% of patients with systemic sarcoidosis. Advances in imaging with cardiac magnetic resonance and positron emission tomography and the increasing recognition of cardiac sarcoidosis has resulted in more patients being suspected of the disease. While the mortality rate in this newer cohort of patients with cardiac sarcoidosis is relatively low, these patients experience high adverse event rates mostly due to fatal ventricular arrhythmic events, and secondarily due to HF. In the current study, fatal ventricular arrhythmia events were observed in 19% of all patients during a median follow-up of 3 years. The findings of this report highlight the importance of risk stratification in patients with cardiac sarcoidosis.
Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Computed Tomography, Magnetic Resonance Imaging, Nuclear Imaging
Keywords: Arrhythmias, Cardiac, Catheter Ablation, Diagnostic Imaging, Heart Failure, Magnetic Resonance Imaging, Cine, Natriuretic Peptide, Brain, Positron-Emission Tomography, Radiofrequency Ablation, Risk Assessment, Sarcoidosis, Stroke Volume, Tachycardia, Ventricular, Ventricular Fibrillation, Ventricular Function, Left
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