Clinical features of cardiac sarcoidosis in a cohort treated with epi- and endocardial VT ablation

Abstract

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Clinical presentation of cardiac sarcoidosis is a chamaeleon. Detection of granuloma in the histopathology proofs the diagnosis of sarcoidosis, however myocardial biopsy is associated with a high sampling error. However, a prompt immune suppressive therapy would influence the patient’s prognosis significantly. Analyzing our cohort of epicardial (and endocardial) VT ablation respectively we intend to identify additional markers supporting the diagnosis sarcoidosis. Method In the last 4 years 130 patients (mean age 49 y, 63 % male ) were hospitalized for epicardial (and endocardial) VT ablation. 19 patients had the diagnosis sarcoidosis or were newly diagnosed. Mean follow up time was 4,3 a. ECG criteria, primary manifestation, histological findings, therapy, history of VT ablation, distribution of scar in MRI and anatomical mapping, PET CT findings, presence of atrial tachycardias, valve disease and comorbidities were documented. Results 6 of 15 (40 %) patients showed right bundle block , 6 of 19 (31 %) had AV nodal disease, 4 patients primarily presented with AV-block III, 14 patients (73 %) primarily presented themselves with ventricular arrhythmias (3 with cardiac arrest), in 93 % patients biopsy revealed elevated CD 68 macrophages and CD 3 T- lymphocytes, 6 of 16 were positive for granuloma (37,5 %). All patients were treated with steroids, 13 patients with a combination of immune suppressive therapy. All were treated with VT ablation, mean 2 ablations per patient. 6 of 6 (100 %) patients showed positive PET CT. Analyzing the scar distribution the most common locations in the 17 segment model were segment 2,3,4 and 5 and septal RV. (septal substrate in 66 %). There was a high correlation between the scar findings in the MRI and the anatomical mapping. No patient died during follow up, 1 patient had high urgent transplant after withdrawal of the steroids. Statistical analysis (Kendall-Tau-b) found a significant correlation between presence of CD3 Lymphocytes, fibrosis, VT as primary manifestation, the presence of ICD, positive PET-CT and septal substrate. Conclusion Patients presenting with ventricular arrhythmias combined with a positive PET CT, elevated CD3 T lymphocytes and CD68 macrophages in the biopsy and septal substrate are suspective of sarcoidosis and immune suppressive therapy should be considered. Further data are warranted to proof that observation and to differentiate from other cardiac diseases.