Cardiac sarcoidosis: diagnosis and management

Abstract

Sarcoidosis is a chronic inflammatory disease of unknown etiology characterized by multi-organ involvement. Endorgan disease consists of granulomatous inflammation, which if left untreated or not resolved spontaneously, leads to permanent fibrosis and end-organ dysfunction. Cardiac involvement and fibrosis in sarcoidosis occur in 5-10% of cases and is becoming increasingly diagnosed. This is due to increased clinical awareness among clinicians and new diagnostic modalities, since magnetic resonance imaging and positron-emission tomography are emerging as "gold standard'' tools replacing endomyocardial biopsy. Despite this progress, isolated cardiac sarcoidosis is difficult to differentiate from other causes of arrhythmogenic cardiomyopathy. Cardiac fibrosis leads to congestive heart failure, arrhythmias and sudden cardiac death. Immunosuppressives (mostly corticosteroids) are used for the treatment of cardiac sarcoidosis. Implantable devices like a cardioverter-defibrillator may be warranted in order to prevent sudden cardiac death. In this article current trends in the pathophysiology, diagnosis and management of cardiac sarcoidosis will be reviewed focusing on published research and latest guidelines. Lastly, a management algorithm is proposed.

Cardiac sarcoidosis: Two case reports

“…8 It also reduces the risk of arrhythmic SCD which is highest in patients with LGE on MRI, history of sustained ventricular arrhythmia, inducible ventricular arrhythmias during electrophysiological studies (EPS), and left ventricular dysfunction. 41,42 Current guidelines therefore recommend ICD implantation in CS patients with sustained VT, post cardiac arrest, and LVEF of 35% or less. Patients with LVEF >35% who have had syncope, evidence of myocardial scar on cardiac MRI or PET scan, indication for permanent pacing, and/or inducible ventricular arrhythmias during EPS are candidates for ICD implantation.…”

“…9 Antiarrhythmic agents including amiodarone may be used to maintain sinus rhythm in patients with atrial arrhythmias or symptomatic VT. 9 In patients with VT refractory to medical therapy, catheter ablation, an effective management option, helps with substantial reduction of total arrhythmia burden. 17,36,42 Cardiac transplantation as a last resort should be considered in CS patients with intractable ventricular arrhythmias or end-stage heart failure. 28…”

Cardiac manifestations of sarcoidosis

“…Cardiac involvement is relatively rare in patients with sarcoidosis and is associated with significant morbidity and mortality. [1,2,3,4,5,6,7,8].…”

Phytosterol Accumulation Results in Ventricular Arrhythmia, Impaired Cardiac Function and Death

“…Cardiac fibrosis is strongly considered as both a cause and a consequence of arrhythmias and HF [4][5][6] . Cardiac fibrosis can manifest in patients with rare inflammatory diseases such as sarcoidosis resulting in congestive HF, cardiac arrhythmias, or both 7 . The severity of cardiac fibrosis correlates with the incidence and progression of both HF and arrhythmias and increases the risk of sudden cardiac death 5,8 .…”