Coronary Artery Ectasia Are Frequently Observed in Patients With Bicuspid Aortic Valves With and Without Dilatation of the Ascending Aorta

Abstract

Background— The presence of coronary artery ectasia (CAE) is influenced by genetic factors and related to the presence of aneurysms in other vascular beds. Bicuspid aortic valve (BAV) disease is frequently accompanied by ascending aortic aneurysm. Because the aortic valve and the proximal parts of the coronary arteries share a common embryonic origin, we hypothesized that CAE is associated with BAV disease. Methods and Results— One hundred seventy-seven patients with suspected aortic valve disease (n=94 BAV, n=83 tricuspid aortic valve) underwent both cardiac magnetic resonance imaging and coronary angiography. To confirm the association of CAE with BAV, the frequency of CAE was evaluated in an in-house BAV registry (n=600, n=231 with available coronary angiogram) and compared with the frequency of CAE in the German Myocardial Infarction (MI) Family Study, in which the heritability of CAE was formerly established (n=899). Furthermore, the frequency of CAE was investigated in an observational registry of real-life patients undergoing coronary angiography for clinically indicated reasons (n=3.097) and in a subgroup of the KORA MI study (Cooperative Health Research in the Region of Augsburg), which is a population-based MI registry (n=403). Compared with tricuspid aortic valve disease, CAE occurred more than twice as frequently in cardiac magnetic resonance–confirmed BAV disease (17% versus 44%; P <0.0001) and CAE was observed similarly often in subjects with BAV with (37%) and without (54%, P =0.11) ascending aortic pathology. The common appearance of CAE in patients with BAV could be independently confirmed in the BAV registry (frequency 37%), whereas CAE was found less frequently in family history of positive MI patients (21%), sporadic MI without familial disposition (10%), and rarely in unrelated real-life catheterization patients (6%). Conclusions— To our knowledge, our data show for the first time that ectatic coronary artery disease is a common appearance of BAV disease with and without ascending aortic ectasia.

Treatment and Outcome of Patients With Coronary Artery Ectasia: Current Evidence and Novel Opportunities for an Old Dilemma

“…The coexistence with other conditions, such as BAV and aortic aneurysms, corroborates the conception of CAE as the manifestation of a systemic disease, in which individual genetical susceptibility might be one of the contributing factors (17,28).…”

“…CAE has been frequently reported in patients with aneurysms in other vascular beds, such as ascending and abdominal aorta and pulmonary arteries (27). Moreover, Meindl et al found that CAE was a common finding in patients with bicuspid aortic valve (BAV), with or without aneurysms of the ascending aorta (28).…”

Clinical indicators and coronary angiographic features of expansive arterial remodelling in patients with abdominal aortic aneurysms

“…In addition, CAE was also frequently observed in patients with ascending aorta aneurysms or bicuspid aortic valves. [11, 12] The major histological features of AAA include chronic medial and adventitial inflammation with medial degeneration, including smooth muscle cell apoptosis and excessive loss of extracellular matrix (ECM), especially extensive elastin fragmentation. [13, 14] Similarly, ectatic coronary arterial walls also demonstrate marked degradation of the medial collagen and elastin fibres, with disruption of the internal and external elastic lamina.…”

“…This approach included the case when the entire artery was conspicuously large without a normal segment. [11] The severity of CAE was determined according to the Markis classification. [8] Diffuse ectasia of 2 or 3 vessels was classified as type I, diffuse disease in 1 vessel and localized disease in another vessel as type II, diffuse ectasia of only 1 vessel as type III, and localized segmental ectasia as type IV.…”

A SOX17-PDGFB signaling axis regulates aortic root development

“…Association of BAV with anomalous coronary arteries has been reported 3 , 4 . Several studies have uncovered significantly increased prevalence of CAAs, such as the absence of the left coronary artery (LCA) in BAV patients compared with their tricuspid counterparts, and BAV patients with coronary anomalies have more postoperative ischemic cardiac events and increased morbidity 5 – 9 . Furthermore, recent genome-wide analysis has identified variant loci in patients with aortic valve stenosis associating with BAV and coronary artery disease, suggesting shared genetic predisposition 10 .…”