Late gadolinium enhancement identified with cardiac magnetic resonance imaging in sarcoidosis patients is associated with long-term ventricular arrhythmia and sudden cardiac death

AIMS: Cardiac involvement with sarcoidosis is a major cause of morbidity and mortality in affected individuals. Cardiac magnetic resonance (CMR) imaging promises a new and more accurate assessment of cardiac sarcoidosis by identifying typical patterns of myocardial fibrosis. We assessed the utility of CMR in the prediction of adverse outcomes. METHODS AND RESULTS: One hundred and six CMR patients with biopsy-proven extracardiac and/or presumed cardiac sarcoidosis were enrolled. Late gadolinium enhancement (LGE) on CMR typical of sarcoidosis was used to determine the presence of cardiac involvement. Clinical endpoints and medical records were assessed and those with implantable cardioverter-defibrillators (ICDs) underwent device interrogation. Survival rates of patients with cardiac sarcoidosis were compared with those with only extracardiac disease. CMR identified 32 (30%) individuals as having cardiac sarcoidosis; the remaining 74 (70%) had only extracardiac disease. At a mean follow-up time of 36.8 ± 20.5 months, patients with cardiac sarcoidosis had a higher rate of the composite cardiac endpoint--comprising sudden cardiac death (SCD) and ventricular tachyarrhythmia--compared with those with only extracardiac disease (P < 0.001). There was a higher rate of SCD or ICD-aborted SCD in patients with cardiac sarcoidosis vs. those without (P = 0.005). In patients with cardiac sarcoidosis, the rate of SCD was lower in those with an ICD compared with those without (P < 0.02). CONCLUSIONS: Patients with evidence of cardiac sarcoidosis on CMR have higher rates of adverse cardiovascular events than those with only extracardiac disease. In patients with sarcoidosis detected on CMR, the presence of an ICD is associated with a lower rate of SCD.