Incidence of bicuspid valve related aortic dissection: a systematic review and meta-analysis

Background: The true incidence of bicuspid valve-related aortic dissection (AD) is extremely difficult to ascertain. This review aimed to provide the reported cumulative incidence of bicuspid aortic valve (BAV)-related AD in actively monitored study populations. Methods: Four electronic databases were used to perform literature searches. A meta-analysis of proportions or means were performed for categorical and continuous variables, as appropriate. Survival data was calculated from the aggregation of Kaplan-Meier (KM) curves from the included studies, where reported. Results: A total of 4,330 patients were identified in eleven studies. A cumulative incidence of bicuspid valve-related AD of 0.6% across a median follow-up time of 9 years was identified. Actuarial survival across this monitored population at 1, 3, 5 and 10 years was 97.2%, 96.7%, 92.45%, and 81.1%, respectively. Conclusions: This systematic review and meta-analysis identified a low incidence of AD across the examined follow-up period. Large, prospective studies involving early identification of bicuspid valve pathology, recruitment, and follow-up of BAV cohorts with comparison to the baseline population are required to most accurately determine the outcomes of these patients.