Long-term outcomes of cardiac sarcoidosis in southern Europe: A 16-year cohort
M Salvador, P M Ventura, J Borges-Rosa, R Silva, L Goncalves, G Costa, M J Vidigal FerreiraAbstract
Background
Sarcoidosis is a granulomatous disease of unknown cause, and cardiac involvement occurs in a minority of patients. Cardiac sarcoidosis (CS) may lead to conduction disturbances, ventricular arrhythmias, heart failure (HF), or even sudden death, though many cases remain asymptomatic.
Purpose
To evaluate the long-term clinical outcomes of patients with suspected CS in a Southern European population over a 16-year follow-up period.
Methods
We reviewed all [18F]FDG-Positron Emission Tomography (PET) reports of patients assessed for suspected sarcoidosis from 2009-2025. Patients with biopsy-confirmed sarcoidosis were evaluated using Hearth Rhythm Society (HRS) criteria to identify probable cardiac involvement. The primary outcome was a composite of HF hospitalization, arrhythmias requiring intervention, pacemaker implantation for complete heart block, and all-cause mortality.
Results
Out of 960 [18F]FDG-PET scans, 314 patients had histologically confirmed extracardiac sarcoidosis. Of these, 27 (8.6%; 41% female; mean age 58.8 ± 13.2 years) were classified as probable cardiac sarcoidosis per HRS criteria, based on [18F]FDG-PET (n = 15), cardiovascular magnetic resonance (CMR) (n = 11), or unexplained LVEF <40% (n = 1). Most patients had no cardiac symptoms (59.3%) during follow-up, and atrial fibrillation occurred in 29.6%, while coronary artery disease and significant valvular disease each occurred in 7.4%. After a median follow-up of 43 [17-104] months, the composite outcome occurred in eleven patients (40.7%): 22.2% had HF hospitalizations; 22.2% had uncontrolled arrythmia requiring intervention (three titration of betablockers due to high burden of premature ventricular complex / non-sustained ventricular tachycardia (VT), two atrial flutter ablation, and one implanted cardiac resynchronization therapy with a defibrillator (CRT-D) due to non-sustained VT and left ventricle systolic disfunction); 14.8% implanted a pacemaker due to complete heart block at a median age of 66 [58-72] years. Additionally, five-patients (18.5%) died during follow-up, two of cardiovascular causes (HF and intestinal ischemia).
Conclusions
Probable cardiac involvement was identified in 8.3% of this Southern European extracardiac sarcoidosis cohort, mostly occurring in asymptomatic patients. Over a median follow-up of 3.6 years, nearly half of the patients experienced major adverse events highlighting the need for vigilant cardiac monitoring in this population.