Clinical presentation, management and prognosis of patients with cardiac sarcoidosis

INTRODUCTION: The course and prognosis of cardiac sarcoidosis (CS) are sparsely described. The purpose of this study was to assess the clinical presentation, treatment response and prognosis for patients with CS.

METHODS: This was a single-centre retrospective study of patients with CS from 2006 to 2016. A total of 197 patients with a sarcoidosis diagnosis were screened, and 17 patients (mean age 46.9 years, 59% men) were diagnosed with CS based on Japanese Ministry of Health and Welfare criteria; 53% were diagnosed by a positive MRI, 29% by endomyocardial biopsy. Of 17 patients, nine (53%) had a left ventricular ejection fraction (LVEF) < 45% at the time of diagnosis. The median follow-up was four years. In 13 patients, an implantable defibrillator was used and six of these (46%) received first appropriate shock therapy after a mean follow-up of two years. A total of 11 (65%) patients were treated with prednisolone and five (45%) of these 11 patients were also treated with another immunosuppressant.

RESULTS: The median LVEF did not change at the last follow-up (p = 0.68), but improved in 30% of patients on combination therapy with prednisolone and proliferation inhibitors, whereas 23% of patients with prednisolone monotherapy experienced further worsening of LVEF. Immunosuppression was not used in 35% of patients. During follow-up, one patient underwent a successful heart transplant, one had a left ventricular assist device implantation and one died from septic shock.

CONCLUSION: In CS patients, ventricular arrhythmias and impairment of LVEF were frequently seen, but the medium-term survival was excellent on heart failure therapy and immunosuppression.

FUNDING: none.

TRIAL REGISTRATION: Danish Data Protection Agency: (File no. RH-2016-301, I-Suite no. 04965).