Characteristics and long-term outcomes of contemporary patients with bicuspid aortic valves

Background We sought to study the impact of surgical intervention on long-term outcomes in bicuspid aortic valve (BAV) patients who develop aortic valvular complications and/or have a concomitant aortopathy. Methods We studied 1890 consecutive patients with BAV (age 50 ± 14 years; 75% men), evaluated in the time period 2003 to 2007. Clinical and imaging data were recorded. The endpoint was a composite of death or type A aortic dissection. Results The mean left ventricular ejection fraction was 55% ± 8%; 31% had New York Heart Association class ≥ III aortic regurgitation, and 17% had mean aortic valve (AV) gradient ≥30 mm Hg. Dilated (≥4 cm) aortic root and ascending aorta were observed in 35% and 42% of patients, respectively. At 8.1 ± 2 years, 918 (49%) patients underwent surgery (883 had AV replacement and/or repair (± combination procedures), 471 with ascending aortic grafting (30 had isolated aortic grafting), and 171 (9%) events (169 deaths and 2 dissections) occurred; 0.4% was the 30-day mortality). On multivariable Cox survival analysis, increasing age (hazard ratio [HR] 1.06, 95% confidence interval [CI] [1.05-1.07]), increasing aortic root size (HR 1.32, 95% CI [1.07-1.65]), decreasing LV ejection fraction (HR 1.04, 95% CI [1.03-1.05]), and hyperlipidemia (HR 1.51, 95% CI [1.20-1.89]) had higher associated events, and surgery related to BAV (time-dependent covariate) (HR 0.44, 95% CI [0.31-0.70]) was associated with significantly lower events (all P <.01). Addition of surgery to BAV risk score (a composite of age, ≥moderate-severe aortic stenosis or regurgitation) and aortic root size further improved risk stratification (the C-statistic increased from 0.65 to 0.73; P =.01). Conclusions Patients with BAV have a high prevalence of AV dysfunction and concomitant aortopathy. Undergoing surgery (AV replacement and/or repair ± AAR) was associated with a significantly lower incidence of death and/or dissection.