title: New Insights Into Unicuspid Aortic Valve Disease in Adults: Not Just a Subtype of Bicuspid Aortic Valves.
abstract: 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.
authors.affiliation: Department of Cardiac Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada.
authors.forename: Pierre-Emmanuel
authors.initials: PE
authors.lastname: Noly
authors.affiliation: Department of Cardiac Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada.
authors.forename: Lauren
authors.initials: L
authors.lastname: Basmadjian
authors.affiliation: Department of Cardiac Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada.
authors.forename: Ismail
authors.initials: I
authors.lastname: Bouhout
authors.affiliation: Department of Cardiology, Hopital Sacre-Coeur de Montreal, University of Montreal, Montreal, Quebec, Canada.
authors.forename: Van Hoai
authors.initials: VH
authors.lastname: Viet Le
authors.affiliation: Department of Cardiac Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada.
authors.forename: Nancy
authors.initials: N
authors.lastname: Poirier
authors.affiliation: Department of Cardiac Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada. Electronic address: i.elhamamsy@icm-mhi.org.
authors.forename: Ismail
authors.initials: I
authors.lastname: El-Hamamsy
mesh_terms: D000328:Adult; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D003937:Diagnosis, Differential; D017548:Echocardiography, Transesophageal / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging* / Q000601:surgery; D006349:Heart Valve Diseases / Q000151:congenital / Q000000981:diagnostic imaging* / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome