Sex Differences and Survival in Adults With Bicuspid Aortic Valves: Verification in 3 Contemporary Echocardiographic Cohorts

Abstract

Background Sex‐related differences in morbidity and survival in bicuspid aortic valve (BAV) adults are fundamentally unknown. Contemporary studies portend excellent survival for BAV patients identified at early echocardiographic‐clinical stages. Whether BAV adults incur a survival disadvantage throughout subsequent echocardiographic‐clinical stages remains undetermined. Methods and Results Analysis was done of 3 different cohorts of consecutive patients with echocardiographic diagnosis of BAV identified retrospectively: (1) a community cohort of 416 patients with first BAV diagnosis (age 35±21 years, follow‐up 16±7 years), (2) a tertiary clinical referral cohort of 2824 BAV adults (age 51±16 years, follow‐up 9±6 years), and (3) a surgical referral cohort of 2242 BAV adults referred for aortic valve replacement (AVR) (age 62±14 years, follow‐up 6±5 years). For the community cohort, 20‐year risks of aortic regurgitation (AR), AVR, and infective endocarditis were higher in men (all P ≤0.04); for a total BAV‐related morbidity risk of 52±4% vs 35±6% in women ( P =0.01). The cohort's 25‐year survival was identical to that in the general population ( P =0.98). AR independently predicted mortality in women ( P =0.001). Baseline AR was more common in men ( P ≤0.02) in the tertiary cohort, with 20‐year survival lower than that in the general population ( P <0.0001); age‐adjusted relative death risk was 1.16 (95% confidence interval [CI] 1.05‐1.29) for men versus 1.67 (95% CI 1.38‐2.03) for women ( P =0.001). AR independently predicted mortality in women ( P =0.01). Baseline AR and infective endocarditis were higher in men (both ≤0.001) for the surgical referral cohort, with 15‐year survival lower than that in the general population ( P <0.0001); age‐adjusted relative death risk was 1.34 (95% CI 1.22‐1.47) for men versus 1.63 (95% CI 1.40‐1.89) for women ( P =0.026). AR and NYHA class independently predicted mortality in women (both P ≤0.04). Conclusions Within evolving echocardiographic‐clinical stages, the long‐term survival of adults with BAV is not benign, as both men and women incur excess mortality. Although BAV‐related morbidity is higher in men in the community, and AR and infective endocarditis are more prevalent in men, women exhibit a significantly higher relative risk of death in tertiary and surgical referral cohorts, which is independently associated with AR.