Background:
Cardiac sarcoidosis (CS) is an inflammatory disease that affects mainly the left ventricle. Involvement of the right ventricle (RV) has also been reported and was associated with unfavourable prognosis. Notably, patients with RV sarcoidosis may be mildly symptomatic or have debilitating, rapidly progressive disease. The severity of right ventricular dysfunction (RVdys) may explain these differences. However, all previous studies focus on the morphological RV changes only.
Objectives:
Our purpose was to evaluate how the presence of RV involvement with and without RVdys can influence the adverse outcomes in patients with CS.
Methods:
We analysed 146 patients from 5 centers in the German CS Registry. RVdys was defined based on reduced Tricuspid Annular Plane Systolic Excursion (TAPSE), presence of tricuspid regurgitation (TR), impaired right ventricular ejection fraction (RVEF), and elevated systolic pulmonary artery pressure (sPAP). Morphological RV involvement (RV morph) was defined as the presence of LGE on CMR, FDG uptake in PET-CT, or both. Occurrence of ventricular arrhythmias, severe AV block, and major adverse events (MACE) defined as death, LVAD implantation, and heart transplantation were assessed in those with and without RVdys.
Results:
Thirty-six patients (25%) had signs of morphological RV involvement (RVmorph+), while 67 individuals (46%) had at least one parameter for RV dysfunction (RVdys+). Rates of MACE rose with increasing numbers of abnormal RV parameters: one (1.3%), nine (13.4%), six (18.8%), five (29.4%) and one (100%) MACE in patients with none, one, two, three and four pathological RVdys parameters, respectively. Incidence of MACE was the highest (15.2%) in the “RVDys+/RVmorph– group”, and the lowest (6.7%) in the “RVDys –/RVmorph+ group”. Ventricular arrhythmias were more frequent in RVmorph+ than RVmorph- group: 15 (43%) vs. 27(25%); P = 0.041. Rates of severe AV block were higher in RVdys+ than in RVdys- patients: 16 (24%) vs. 10 (13%); P = 0.07.
Conclusion:
Right ventricular dysfunction in CS is frequent and not always accompanied by abnormal CMR and the PET scans. The severity of RVdys was associated with more adverse events. On the other hand, ventricular arrhythmias were more frequent in patients with LGE or abnormal PET, independent from the RV dysfunction. RV structural changes and dysfunction should be considered as complementary, rather than interchangeable features when determining the prognosis of CS.
