Cardiac sarcoidosis; update for the heart failure specialist.

Abstract

Purpose of review: This review presents contemporary data on epidemiology, common presentations, investigations and diagnostic algorithms, treatment and prognosis. It particularly focuses on topics of most relevance to heart failure specialists, including what left ventricle (LV) function changes can be expected after treatment and outcomes to all standard and advanced heart failure therapies.

Recent findings: Around 5% of sarcoidosis patients have clinically manifest cardiac sarcoidosis (CS), presenting with significant arrhythmias (such as conduction disturbances and ventricular arrhythmias) or newly developed unexplained heart failure. These cardiac symptoms (including sudden cardiac death) may be the initial manifestations of CS. While cardiac magnetic resonance imaging (CMR) is the preferred method for identifying fibrosis in the myocardium, FDG-positron emission tomography (FDG-PET) helps in identifying active inflammation within the myocardium and aids in managing immunosuppressive treatment. The concept of isolated CS is much debated. However very importantly, recent data have shown that some patients diagnosed with 'clinically and imaging isolated CS' are subsequently found to have genetic cardiomyopathy. The management of CS involves a comprehensive approach including medications for immunosuppression, all standard heart failure medication and, in high-risk patient's implantable cardioverter defibrillators (ICDs). In CS patients with terminal heart failure who do not respond to medical and surgical interventions, heart transplantation and ventricular assist devices should be considered. Long-term results after transplantation are generally favorable and comparable to non-CS patients. The degree of left ventricular dysfunction remains a crucial prognostic factor in CS cases. Outcomes for CS have very significantly improved, over the last two decades due to earlier diagnosis, advanced heart failure treatments, and the strategic use of ICD therapy.

Summary: Outcomes for CS have significantly improved, over the last two decades due to earlier diagnosis, advanced heart failure treatments, and the strategic use of ICD therapy.