941 sarcoidosis: the importance of multimodality imaging

Abstract A 55-years old sporty man, without any cardiovascular risk factors nor previously known cardiological history, presented for a cardiological evaluation due to extra-systolic palpitations and dyspnea. The ECG documented a new onset of right bundle branch block, left anterior fascicular block and first degree of atrio-ventricular block. Echocardiography revealed severe left ventricular (LV) systolic dysfunction (LV ejection fraction -EF- 34%), showing also right ventricular (RV) dilatation and disfunction. A 24-hour Holter monitoring did not show any significant arrhythmias and maximal stress test did not reveal any ECG changes nor arrhythmias. Cardiac magnetic resonance (CMR) confirmed the presence of severe biventricular dysfunction; multiple areas of edema and late gadolinium enhancement (LGE) were observed, with ischemic and nonischemic pattern and extensive involvement of the RV and the interventricular septum. In the suspicion of cardiac sarcoidosis a 18-fluorodeoxyglucose positron emission tomography (PET) was performed, confirming the presence of an inflammatory cardiomyopathy in an active phase. A subsequent endomyocardial biopsy was performed, which demonstrated the presence of non-caseating granulomas, signs of inflammation and fibrosis, consistent with the diagnosis of cardiac sarcoidosis. No signs of extra-cardiac involvement were present. The patient started anti-inflammatory therapy with Prednisone and Metotrexate, along with anti-neurohormonal therapy. However, due to a significant increase in ventricular arrhythmic burden, an implantable cardioverter defibrillator was placed during early follow-up, and Amiodarone therapy was started. After 5 months of medical therapy, PET scan showed a marked reduction of cardiac inflammation and echocardiography showed a significant LVEF improvement (from 34% to 43%). After slow tapering of steroid therapy Prednisone was stopped, whereas Metotrexate, Amiodarone and anti-neurohormonal therapy were maintained. Follow-up is still ongoing without clinical events. Sarcoidosis is a systemic inflammatory disease characterized by the presence of non-caseating granulomas in multiple organs. Cardiac involvement is associated with higher incidence of heart failure, ventricular arrhythmias and all-cause mortality. Isolated cardiac involvement is rare but associated with worse prognosis. Multimodality imaging is of paramount importance for the diagnosis and monitoring therapy.