PO-05-148 VENTRICULAR DYSFUNCTION IN SARCOIDOSIS CARDIOMYOPATHY: INFLAMMATION IS NOT ALWAYS THE ONE TO BLAME

Abstract

Clinical presentation A 63-year-old female was admitted after repeated syncopal events. She had a previous history of type 1 diabetes and hypertension. ECG on admission showed sinus rhythm with prolonged PR interval (320ms) and normal QRS duration. Imaging work up Coronary angiography revealed patent arteries. Echocardiography showed regional wall motion abnormalities but preserved left ventricular ejection fraction (LVEF). Cardiac magnetic resonance displayed patchy contrast retention following a non-coronary distribution. Whole-body positron emission tomography showed intense, multifocal tracer uptake in the myocardium and in the thoracic lymph nodes, in a pattern consisting with sarcoidosis. Diagnosis and Initial Treatment A clinical diagnosis of cardiac sarcoidosis (CS) was made. Following the implantation of a biventricular defibrillator, she was discharged from the hospital, and systemic immunosuppression with 30 mg prednisone daily was initiated. Follow-up Six months after diagnosis, interval PET showed reduction in the degree of myocardial inflammation, and no major change in the extent of myocardial scarring as based on myocardial perfusion (Table 1). Nevertheless, LVEF had dropped from 53% to 36%. Device interrogation revealed a high burden of PVCs. PVC-mediated cardiomyopathy was suspected, and amiodarone was initiated. Also because of uncertainty for the precipitous decline of LVEF and concern that it was multifactorial, additional immunosuppression with infliximab was instituted. After four months with amiodarone (but only one dose of infliximab), the PVC burden was reduced to 1%, biventricular pacing improved to target, and LVEF was restored to normal. These outcomes are maintained two years on. Interpretation