Outcomes in Adults With Bicuspid Aortic Valves

CONTEXT: Bicuspid aortic valve is the most common congenital cardiac anomaly in the adult population. Cardiac outcomes in a contemporary population of adults with bicuspid aortic valve have not been systematically determined. OBJECTIVE: To determine the frequency and predictors of cardiac outcomes in a large consecutive series of adults with bicuspid aortic valve. DESIGN, SETTING, AND PARTICIPANTS: Cohort study examining cardiac outcomes in 642 consecutive ambulatory adults (mean SD age, 35 16 years; 68% male) with bicuspid aortic valve presenting to a Canadian congenital cardiac center from 1994 through 2001 and followed up for a mean (SD) period of 9 (5) years. Frequency and predictors of major cardiac events were determined by multivariate analysis. Mortality rate in the study group was compared with age- and sex-matched population estimates. MAIN OUTCOME MEASURES: Mortality and cause of death were determined. Primary cardiac events were defined as the occurrence of any of the following complications: cardiac death, intervention on the aortic valve or ascending aorta, aortic dissection or aneurysm, or congestive heart failure requiring hospital admission during the follow-up period. RESULTS: During the follow-up period, there were 28 deaths (mean SD, 4% 1%). One or more primary cardiac events occurred in 161 patients (mean SD, 25% 2%), which included cardiac death in 17 patients (mean SD, 3% 1%), intervention on aortic valve or ascending aorta in 142 patients (mean SD, 22% 2%), aortic dissection or aneurysm in 11 patients (mean SD, 2% 1%), or congestive heart failure requiring hospital admission in 16 patients (mean SD, 2% 1%). Independent predictors of primary cardiac events were age older than 30 years (hazard ratio HR, 3.01; 95% confidence interval CI, 2.15-4.19; P<.001), moderate or severe aortic stenosis (HR, 5.67; 95% CI, 4.16-7.80; P<.001), and moderate or severe aortic regurgitation (HR, 2.68; 95% CI, 1.93-3.76; P<.001). The 10-year survival rate of the study group (mean SD, 96% 1%) was not significantly different from population estimates (mean SD, 97% 1%; P = .71). At last follow-up, 280 patients (mean SD, 45% 2%) had dilated aortic sinus and/or ascending aorta. CONCLUSIONS: In this study population of young adults with bicuspid aortic valve, age, severity of aortic stenosis, and severity of aortic regurgitation were independently associated with primary cardiac events. Over the mean follow-up duration of 9 years, survival rates were not lower than for the general population.