title: Diagnosis of congenital unicuspid aortic valve in adult population: the value and limitation of transesophageal echocardiography.
abstract: BACKGROUND
  This study aimed to assess the accuracy of two-dimensional echocardiography (echo) in diagnosing unicuspid aortic valve (UAV) and to determine echo features that could improve the diagnosis.
  
  
  METHODS
  We reviewed transthoracic/transesophageal echoes (TTE/TEE) from our hospital database for adult patients who had aortic valve surgery with a preoperative echo diagnosis of UAV or equivocal diagnosis of bicuspid aortic valve (BAV) BAV/UAV. Morphological characteristics of AV and ascending aortic dimensions were evaluated.
  
  
  RESULTS
  Nineteen patients were identified, 13 (11 Male, 2 Female, mean age 47 ± 10 years) had surgically confirmed diagnosis of UAV, six had BAV. The incidence of UAV was 2.6%. For diagnosing UAV, the sensitivity and specificity of TTE was 27% and 50% and those of TEE was 75% and 86%, respectively. For TTE, positive predictive value (PPV) was 60% and negative predictive value (NPV) was 20%. By TEE, PPV was 90% and the NPV was 67%. In UAV patients, 85% had severe aortic stenosis (mean gradient 45 ± 16 mmHg, AVA: 0.9 ± 0.2 cm²). 46% had ascending aorta aneurysm (mean aortic root, sinutubular junction, ascending aorta dimensions: 36 ± 3 mm, 31 ± 4 mm and 41 ± 8 mm). Patients with ascending aortic aneurysm were younger (41 ± 11 years vs. 52 ± 5 years, P < 0.05) All UAV were unicommissural with a posteriorly positioned commissural attachment, 69% were heavily calcified. Diagnostic accuracy was limited by quality of images, severity, and distribution of calcification.
  
  
  CONCLUSIONS
  TEE is the diagnostic modality of choice in UAV. Identifying several echo features may improve its diagnostic accuracy.
authors.affiliation: Cardiac Ultrasound Laboratory, Department of Cardiology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA. john.chu@healthotago.co.nz
authors.forename: John W
authors.initials: JW
authors.lastname: Chu
authors.forename: Michael H
authors.initials: MH
authors.lastname: Picard
authors.forename: Arvind K
authors.initials: AK
authors.lastname: Agnihotri
authors.forename: Michael G
authors.initials: MG
authors.lastname: Fitzsimons
mesh_terms: D000328:Adult; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D017548:Echocardiography, Transesophageal / Q000379:methods*; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity