Outcomes in Adults With Bicuspid Aortic Valves

Abstract

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; PϽ.001), moderate or severe aortic stenosis (HR, 5.67; 95% CI, 4.16-7.80; PϽ.001), and moderate or severe aortic regurgitation (HR, 2.68; 95% CI, 1.93-3.76; PϽ.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.

Risk factors for aortic valve disease in bicuspid aortic valve: A family‐based study

“…There have been conflicting reports regarding the association between BAV morphology and AVD [Fernandes et al, 2004;Tzemos et al, 2008]. Fernandes et al [2004] identified an association between RN BAV and AVD in a pediatric population, while Tzemos et al [2008] found no association in an adult population . Despite clear evidence that BAV is a heritable condition with a recurrence risk of approximately 8%, it remains unclear whether BAV morphology is also genetically determined and whether it predicts the development of AVD [Cripe et al, 2004;Fernandez et al, 2009;Hinton et al, 2009].…”

“…AVD (stenosis and/or insufficiency) typically manifests later in life, affecting more than 2% of the population, and remains a significant surgical problem with approximately 100,000 valve replacement procedures performed each year in the US [Bonow et al, 2006;Nkomo et al, 2006;Otto, 2006;Rosamond et al, 2008;Lloyd-Jones et al, 2009]. The majority of AVD cases at any age have an underlying BAV, and longitudinal studies in young adults with BAV have shown that >20% ultimately develop AVD requiring intervention [Tzemos et al, 2008]. Two patterns of BAV morphology are commonly observed; $70% of cases have fusion of the right and left (RL) coronary cusps with the remainder consisting almost entirely of those with fusion of the right and non (RN) coronary cusps [Ward, 2000;.…”

“…Two patterns of BAV morphology are commonly observed; $70% of cases have fusion of the right and left (RL) coronary cusps with the remainder consisting almost entirely of those with fusion of the right and non (RN) coronary cusps [Ward, 2000;. There have been conflicting reports regarding the association between BAV morphology and AVD [Fernandes et al, 2004;Tzemos et al, 2008]. Fernandes et al [2004] identified an association between RN BAV and AVD in a pediatric population, while Tzemos et al [2008] found no association in an adult population .…”

Bicuspid aortic valve endocarditis complicated by perivalvular abscess

“…As BAV is one of the most common congenital cardiac abnormalities (11)(12)(13), the frequency of cardiac and vascular events associated with BAV might be greater than the combined cardiovascular morbidity of all other congenital heart diseases (2). High rates of cardiovascular adverse events in adults with BAV have been recently reported by two large-scale studies (14,15).…”

“…In a cohort of 310 patients with aortic valve IE (1), a BAV was identified in 16% of cases. In a study including 642 adults with BAV, IE occurred in 13 patients over 9 years of follow-up (15), while in another study, 4 out of 212 patients with BAV developed IE during a follow-up of 20 years (14). A recent multicentre study (1) demonstrated that of 148 patients with BAV, 4(2.7%) were diagnosed with IE.…”