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Nikolaos Tzemos, MD; Judith Therrien, MD; James Yip, MD; George Thanassoulis, MD; Sonia Tremblay, MD; Michal T. Jamorski, BSc; Gary D. Webb, MD; Samuel C. Siu, MD, SM

JAMA. 2008;300(11):1317-1325.

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.

Author Affiliations: Peter Munk Cardiac Centre and Toronto Congenital Cardiac Centre for Adults, University Health Network, University of Toronto, Toronto, Ontario, Canada (Drs Tzemos and Siu and Mr Jamorski); Department of Cardiology, National University Hospital, Singapore (Dr Yip); Sir M. B. Davis Jewish General Hospital, Department of Medicine, McGill University, Montreal, Quebec, Canada (Drs Therrien, Thanassoulis, and Tremblay); Philadelphia Adult Congenital Heart Center, Children's Hospital of Philadelphia, Department of Medicine, University of Pennsylvania, Philadelphia (Dr Webb); and Division of Cardiology, University of Western Ontario, London, Ontario, Canada (Dr Siu).

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