1Justus-Liebig-Universität Giessen Kardiologie und Angiologie Gießen, Deutschland; 2Universitätsklinikum Leipzig Klinik und Poliklinik für Kardiologie Leipzig, Deutschland; 3Herzzentrum Leipzig - Universität Leipzig Rhythmologie Leipzig, Deutschland; 4Universitätsklinikum Gießen und Marburg GmbH Medizinische Klinik I - Kardiologie und Angiologie Gießen, Deutschland
Background: Cardiac sarcoidosis (CS) can manifest with variety of unspecific symptoms. It can mimic all types of cardiomyopathies but most often it resembles dilated cardiomyopathy. Involvement of right ventricle (RV) is rare and may lead to a false diagnosis of arrhythmogenic right ventricular cardiomyopathy and delay of the immunosuppressive therapy. The aim of this research was to study the characteristics and the outcomes of this peculiar subtype of patients.
Methods: We analyzed 112 patients diagnosed with CS after elaborate work-up with cardiac magnetic resonance (CMR), FDG PET and endomyocardial biopsy (EMB). All patients underwent follow-up for occurrence of combined endpoint of death, LV assist device implantation or heart transplantation. Patients with evidence of late gadolinium enhancement (LGE) involving RV (RV group) were identified and compared to the rest (DCM group) for baseline clinical characteristics, arhythmic outcomes and combined endpoint.
Results: Involvement of the RV in CMR was observed in 19 (17%) patients. Patients in the RV group were more frequently male: 84% vs. 57%, P = 0.032. EMB confirmed CS in 63% vs. 19.4% in the DCM group; P= 0.0001. LV ejection fraction in the RV group was comparable to the DCM group: 42±14% vs. 45±14%; P = 0.3. Furthermore, in the RV group the rates of sustained ventricular tachycardia were significantly higher than the DCM: 68% vs. 34%; P = 0.009. Long-term immunosuppressive therapy was started in 74% in RV and in 70% of the DCM group; P=0.77. Despite this, during follow-up, the rate of death, LVAD implantation or heart transplantation was significantly higher in the RV than in the DCM group; 26.3% vs. 7.5%; P = 0.03. In binary logistic analysis, the OR for the combined endpoint was 4.4; 95% CI 1.2-15.7.
Conclusions: Patients with CS and RV involvement in CMR have worse outcomes as compared to patients presenting as DCM, despite the comparable LV EF and immunosuppressive therapy.