Adverse Outcomes With Cardiac Sarcoid: New Zealand Experience in the Modern Era

Abstract

Diagnosis of cardiac sarcoidosis (CS) has increased with availability of multi-modal imaging yet presenting phenotype and natural history of the disease remain highly variable. This study evaluated experience in the Auckland/Northland regions between 2004 and 2022. A retrospective observational study of patients with CS defined according to Heart Rhythm Society (HRS) criteria. Demographics, diagnostic presentation, and adverse clinical outcomes were evaluated, including heart failure, ventricular arrhythmia (VA), device therapy, transplant, and death. Ninety-nine patients met diagnostic criteria for CS; median age was 55±11 years and 52% were male. Clinical presentations included symptomatic conduction disease in 53%, new heart failure in 24%, in VA 16%, and incidental in 6%. Diagnosis was confirmed by imaging studies: cardiac MRI in 85 patients (86%) and/or positive computed tomography-positron emission tomography (CT-PET) in 47 (47%), and positive cardiac biopsy in 24 (24%). Devices were implanted in 84 (85%) patients, with defibrillator capability in 67 (80%). Seventy (71%) patients experienced 158 adverse events: death in 13%, heart failure in 28%, VA in 27%, device therapies in 21%, progressive conduction disease in 7%, atrial arrhythmias in 3%, stroke in 1%, and transplant in 6%. Twenty-two patients (26%) did not meet Class I indications for implantable cardioverter defibrillator, but more than one-third required device therapy. A regimen of high-dose steroid (>30 mg prednisone) with a tapering regimen was used in 65% of patients, with additional steroid sparing agents in 55%. Complete disease suppression was assessed by repeat PET at 3 months was observed in 55%. Adverse cardiovascular events are common with CS. Over 50% of patients experience cardiac arrhythmia and consideration of appropriate device therapy is important at diagnosis.