Background
Cardiac Behçet’s syndrome (cardiac BS) is rare but lethal. And the influencing factors of prognoses in cardiac BS are not clear. This study was conducted to summarize the features of cardiac BS and find the predictors of unfavourable prognoses for cardiac BS patients.
Methods
Sixty-six cardiac BS patients were included from 1467 BS patients in the Shanghai Behçet’s syndrome database. The median follow-up duration was 4.0 (2.0–7.0) years. The unfavourable prognosis was defined as a compound event containing all-cause death and rehospitalization related to worsened cardiac lesions or postoperative complications. Logistic regression analyses were performed to evaluate the predictors of unfavourable prognoses.
Results
In this study, the mortality rate of cardiac BS patients was 10.61% (7/66), and 22 (33.33%) patients experienced two or more surgeries. Ninety-six cases were collected from 66 cardiac BS patients. The effective rates of postoperative biological agents and Janus kinases (JAK) inhibitors in cardiac BS patients were 88.89% (16/18) and 81.25% (13/16) respectively. Former smoking, valve replacement or repairment and poor compliance were found to be the independent risk factors of unfavourable prognoses; preoperative immunosuppressive therapies and postoperative immunosuppressive therapies were independent protective factors for unfavourable prognoses in total cardiac BS. In 65 cases of cardiac BS after aortic valve surgeries, former smoking was an independent risk factor for unfavourable prognoses. Bentall procedure, postoperative traditional DMARDs, postoperative JAK inhibitors or biological agents were independent protective factors for unfavourable prognoses in cardiac BS patients after aortic valve surgeries.
Conclusion
Both preoperative immunosuppressive therapies and postoperative immunosuppressive therapies are necessary for cardiac BS patients. Bentall procedure is recommended to be performed in BS patients with severe AR. Besides biological agents, JAK inhibitors can be used in cardiac BS patients after aortic valve surgeries for promoting favourable outcomes.