Incidence of Aortic Complications in Patients With Bicuspid Aortic Valves

Abstract

Bicuspid aortic valve is the most common congenital heart defect,¹ affecting 1.3% of the population.² Its most common complication is valve dysfunction.^3,4 However, BAV has also been suggested to cause aortopathy,^5-7 a process touted as similar to that of Marfan syndrome with independent aortic dilatation.^3-5,7,8 Aortic dissection is the most serious complication due to its high mortality.⁹ Necropsy studies suggest that these patients are at considerable risk of aortic dissection^10,11 and high attributable risk of dissection is noted for BAV.^6,12 Thus, it is estimated that BAV is responsible for more deaths than all other congenital heart defects combined.¹³Consequently, carriers live under the threat of sudden death.¹⁴ These associations are now challenged by 2 recent studies in which few dissections were observed^3,4; we reported outcomes in a community-cohort limited to healthy patients with normally functioning BAV³ and others reported on a tertiary-referral-center cohort with relatively short follow-up.⁴ Thus, aortic dissections occurring late after diagnosis or in patients with significant BAV dysfunction may have eluded ascertainment. Furthermore, aortic dissection may occur in patients with undiagnosed BAV or uncertain bicuspid diagnosis. Hence, a population-based study with comprehensive ascertainment of aortic events among patients with BAV is lacking. Olmsted County, Minnesota, provides a geographically defined population with few clinicians or hospitals delivering health care in the community¹⁵: all echocardiograms are reviewed by one laboratory, and all cardiovascular surgeries are performed at the same center. Our objective was to determine the incidence of aortic complications among patients with the condition in the community as well those in the general BAV population.