New Insights Into Unicuspid Aortic Valve Disease in Adults: Not Just a Subtype of Bicuspid Aortic Valves

Abstract

BackgroundThe clinical presentation, echocardiographic features, and patterns of aortic dilatation in unicuspid aortic valves (UAVs) are not well defined. MethodsFrom 2011 to 2015, all data from adult patients with confirmed UAVs (n = 42) who underwent aortic valve surgery were reviewed. ResultsThe 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 cm2. 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%). ConclusionsUAVs 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.