Clinical profile and mortality predictors in cardiac sarcoidosis: A single centre observational study

Background Cardiac sarcoidosis (CS) may present with conduction abnormalities, ventricular arrhythmias, and heart failure. The delay in recognizing cardiac involvement in systemic sarcoidosis leads to disease progression, resulting in major morbidity and mortality. We studied the clinical, electrocardiographic, and imaging features of cardiac sarcoidosis and its mortality predictors. Methods The clinical data of patients with CS who presented to the Sree Chitra Tirunal Institute for Medical Sciences and Technology between 2005 and 2021 were retrospectively analysed. The diagnosis of CS was based on the 2014 Heart Rhythm Society Expert Consensus recommendations and the 2016 Japanese Circulation Society clinical diagnosis criteria. Patients with obstructive coronary artery disease and possible myocarditis were excluded from the study. Results Forty-three patients of CS (31 males), aged 49 (8.8) years, were followed up for a mean duration of 4.3 (range 1.87-6.5) years. The presenting clinical manifestations were ventricular tachycardia (VT) 14/43 (33%), acute heart failure 14/43 (33%), complete heart block 10/43 (23%), and non-sustained VT/symptomatic ventricular premature complexes 2/43 (5%). Systemic manifestations included lymphadenopathy 28/43 (65%), pulmonary parenchymal involvement 26/43 (60%), and neurological involvement 8/43 (19%). The mean basal left ventricular ejection fraction at presentation was 41.1% (standard deviation 16.1%), and 31/33 (94%) of the patients had late gadolinium enhance-ment in cardiac MRI, with the predominant pattern being sub-epicardial 18/33 (58%) or mid-myocardial 17/33 (54%). Eighteen (42%) patients received implantable cardioverter defibrillator (ICD); nearly half had appropriate ICD shocks. On follow-up, 11 (25%) patients died, 10 (23%) had recurrent heart failure admissions, and 5 (29%) had recurrent ICD shocks. Multivariate analysis revealed higher New York Heart Association (NYHA) class/clinical heart failure at presentation, elevated erythrocyte sedimentation rate at diagnosis, and persistent low ejection fraction during follow-up to be predictors of mortality, not VT. Survival analysis showed that recurrent heart failure admissions predict early mortality. Conclusion Although arrhythmia was the most common manifestation, clinical heart failure was seen in nearly half of the patients with a diagnosis of CS. A high prevalence of heart failure, along with 25% mortality in our study, may indicate a delayed recognition of cardiac involvement in these patients' natural history. Recurrent heart failure admissions predicted early mortality.