Bicuspid aortic valve (BAV) is the most common congenital heart defect, affecting 1.3% of the population.  Although it is now well recognised that having a bicuspid aortic valve can lead to early valve dysfunction,  the association between BAV and aortic pathology has remained less clear.

The aim of this retrospective cohort study was to assess the incidence of aortic complications in a cohort of patients with BAV.  Long-term follow-up of patients diagnosed with definite BAV by echocardiography from 1980 to 1999 was studied and the authors also searched for aortic complications of patients whose bicuspid valves had gone undiagnosed.  The main outcome measure was the incidence of thoracic aortic dissection, ascending aortic aneurysm, or aortic surgery.

The cohort included 416 consecutive patients with bicuspid aortic valve diagnosed by echocardiography, with a mean follow-up of 16 years (6530 patient-years).  Aortic dissection occurred in 2 of 416 patients (age-adjusted relative-risk 8.4; P=.003) compared with the general population. Aortic dissection incidences for patients 50 years or older at baseline and bearers of aortic aneurysms at baseline were 17.4 and 44.9 cases per 10 000 patient-years,respectively.  A comprehensive search for aortic dissections in undiagnosed bicuspid valves revealed 2 additional patients, allowing estimation of aortic dissection incidence in bicuspid valve patients irrespective of diagnosis status.  Of 384 patients without baseline aneurysms, 49 developed aneurysms at follow-up, incidence of 84.9 cases per 10 000 patient-years and an age-adjusted relative risk 86.2 (P.001 compared with the general population). The 25-year rate of aortic surgery was 25% (95% CI, 17.2%-32.8%).

Conclusions:

This study found that in patients with bicuspid aortic valve the incidence of aortic dissection is low but significantly higher than that of the general population.