Contemporary natural history of bicuspid aortic valve disease: a systematic review.

We performed a systematic review of the current state of the literature regarding the natural history and outcomes of bicuspid aortic valve (BAV). PubMed and the reference lists of the included articles were searched for relevant studies reporting on longitudinal follow-up of BAV cohorts (mean follow-up ≥2 years). Studies limited to patients undergoing surgical interventions were excluded. 13 studies (11 502 patients with 2-16 years of follow-up) met the inclusion criteria. There was a bimodal age distribution (30-40 vs ≥50 years), with a 3:1 male to female ratio. Complications included moderate to severe aortic regurgitation (prevalence 13%-30%), moderate to severe aortic stenosis (12%-37%), infective endocarditis (2%-5%) and aortic dilatation (20%-40%). Aortic dissection or rupture was rare, occurring in 38 patients (0.4%, 27/6446 in native BAV and 11/2232 in post). With current aggressive surveillance and prophylactic surgical interventions, survival in three out of four studies was similar to that of a matched general population. In this systematic review, valvular dysfunction warranting surgical intervention in patients with BAV were common, aortic dissection was rare and, with the current management approach, survival was similar to that of the general population.