Abstract
Objective: The combination of aortic coarctation (CoA) and bicuspid aortic valve (BAV) is assumed to be associated with a higher risk of ascending aortic dilatation and type A dissection, and current European Society of Cardiology (ESC) guidelines advise therefore to operate at a lower threshold in the presence of CoA. The aim of our study is to evaluate whether the coexistence of CoA in BAV patients is indeed associated with a higher risk of ascending aortic events (AAE). Methods: In a retrospective study, all adult BAV patients visiting the outpatient clinic of our tertiary care center between February 2003 and February 2019 were included. The primary end point was an ascending aortic event (AAE) defined as ascending aortic dissection/rupture or preventive surgery. The secondary end points were aortic dilatation and aortic growth. Results: In total, 499 BAV patients (43.7% female, age 40.3 ± 15.7 years) were included, of which 121 (24%) had a history of CoA (cBAV). An aortic event occurred in 38 (7.6%) patients at a mean age of 49.0 ± 13.6 years. In the isolated BAV group (iBAV), significantly more AAE occurred, but this was mainly driven by aortic valve dysfunction as indication for aortic surgery. There was no significant difference in the occurrence of dissection or severely dilated ascending aorta (>50 mm) between the iBAV and cBAV patients (p = 0.56). The aortic diameter was significantly smaller in the cBAV group (30.3 ± 6.9 mm versus 35.7 ± 7.6 mm; p < 0.001). The median aortic diameter increase was 0.23 (interquartile range (IQR): 0.0–0.67) mm/year and was not significantly different between both groups (p = 0.74). Conclusion: Coexistence of CoA in BAV patients was not associated with a higher risk of aortic dissection, preventive aortic surgery, aortic dilatation, or more rapid aorta growth. This study suggests that CoA is not a risk factor in BAV patients, and the advice to operate at lower diameter should be reevaluated.
Highlights
Bicuspid aortic valve (BAV) is the most common congenital heart defect with a prevalence of 0.5–1.3% [1]
In a pediatric cohort of BAV patients, CoA was associated with smaller ascending aortic diameters, while in adults, contradicting studies about CoA in BAV patients have been published, with some showing a higher risk of ascending aortic events (AAE) when CoA was present in these patients, while others found no relation [4,7,8]
The aim of this study is to investigate whether the coexistence of CoA in BAV patients is associated with ascending aortic events (AAE), aortic dilatation and aortic diameter increase
Summary
Bicuspid aortic valve (BAV) is the most common congenital heart defect with a prevalence of 0.5–1.3% [1]. In about half of all BAV patients, ascending aortic dilation develops, predisposing for life-threatening complications such as aortic dissection [2]. BAV can occur isolated (iBAV), and in combination with additional heart defects or in the context of a syndrome. Depending on age groups studied, the prevalence of aortic coarctation (CoA) in BAV patients varies between 22% and 36% and is increased in younger age groups [2,3,4,5]. After CoA correction, many patients suffer from hypertension, which is a known risk factor for aortic dissection and higher mortality, especially in BAV patients [4,6]. In a pediatric cohort of BAV patients, CoA was associated with smaller ascending aortic diameters, while in adults, contradicting studies about CoA in BAV patients have been published, with some showing a higher risk of ascending aortic events (AAE) when CoA was present in these patients, while others found no relation [4,7,8]
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