Background: The clinical presentation, echocardiographic features, and patterns of aortic dilatation in unicuspid aortic valves (UAVs) are not well defined.
Methods: From 2011 to 2015, all data from adult patients with confirmed UAVs (n = 42) who underwent aortic valve surgery were reviewed.
Results: The mean age of the study population was 33.9 ± 1.7 years; 33 were male (78%). Mixed aortic valve disease (stenosis and regurgitation) was the most common mode of presentation (n = 25; 59%). Preoperative diagnosis of UAV was made in only 6 patients (14%). Preoperative transesophageal echocardiography in the operating room increased the diagnosis to 69% of the patients. The peak and mean aortic gradients were 80 ± 29 mm Hg and 50 ± 20 mm Hg, respectively. The aortic valve area was 1.1 ± 0.4 cm(2). Overall, 21% of patients (n = 9) had aortic dilatation (> 45 mm): 6 patients (14%) had isolated ascending aortic dilatation, 1 patient (2%) had isolated aortic root dilatation, and 2 patients (5%) had combined aortic root and ascending aortic dilatation. In contrast, 71% of patients (n = 30) had a dilated aortic annulus (> 25 mm). Mean aortic diameters measured using transesophageal echocardiography were: aortic annulus: 25.8 ± 3.4 mm; sinus of Valsalva: 32.1 ± 5 mm, sinotubular junction: 28.1 ± 5 mm, and ascending aorta: 36.2 ± 5 mm. The valve was repaired using a bicuspidization technique in 8 patients (19%) and replaced using a Ross procedure in 34 patients (81%).
Conclusions: UAVs remain significantly underdiagnosed in young adults with aortic valve disease. UAVs are associated with a distinct pattern of aortic dilatation, which is mainly present at the level of the aortic annulus. In a small proportion of patients, the valve can be surgically repaired.
Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.