title: Left ventricular noncompaction in patients with bicuspid aortic valve.
abstract: BACKGROUND
  Left ventricular noncompaction (LVNC) is commonly associated with complex congenital anomalies. The association of LVNC with less complex but more frequent anomalies, such as bicuspid aortic valve (BAV), is not well described in the literature. The aims of this study were to (1) determine the incidence of association of LVNC with the most common congenital anomaly, BAV, in an echocardiographic database and (2) describe clinical and imaging characteristics of these patients.
  
  
  METHODS
  An echocardiography database was retrospectively interrogated to identify 109 patients who fulfilled the echocardiographic criteria for BAV from July 1, 2011, to March 31, 2013. Echocardiograms were carefully evaluated to identify patients with concomitant LVNC.
  
  
  RESULTS
  Twelve patients (11.0%) with BAV fulfilled the criteria for LVNC. The mean age at diagnosis was 33 ± 16.9 years; nine of 12 were men. Eight patients (66.7%) had symptoms during initial presentation. The most common BAV morphology was fusion of the right and left coronary cusps. Nine patients had mild or moderate aortic valve dysfunction (aortic regurgitation and/or stenosis), and eight had associated aortopathy. LVNC was located at the apex in all patients except one. Mean systolic global longitudinal strain was -16.9 ± 2.7%.
  
  
  CONCLUSIONS
  In this series of patients, concomitant BAV and LVNC were observed in 11% of a BAV population. Further studies are needed to understand the genetic and pathophysiologic basis of this association.
authors.affiliation: Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, Milwaukee, Wisconsin.
authors.forename: Anushree
authors.initials: A
authors.lastname: Agarwal
authors.forename: Bijoy K
authors.initials: BK
authors.lastname: Khandheria
authors.forename: Timothy E
authors.initials: TE
authors.lastname: Paterick
authors.forename: Shannon C
authors.initials: SC
authors.lastname: Treiber
authors.forename: Michelle
authors.initials: M
authors.lastname: Bush
authors.forename: A Jamil
authors.initials: AJ
authors.lastname: Tajik
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging; D000082882:Bicuspid Aortic Valve Disease; D015897:Comorbidity; D004452:Echocardiography / Q000706:statistics & numerical data*; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D006349:Heart Valve Diseases / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D017678:Sex Distribution; D014922:Wisconsin / Q000453:epidemiology; D055815:Young Adult
keywords: BAV; Bicuspid aortic valve; C; CMR; Cardiac magnetic resonance; Compacted; LV; LVNC; Left ventricular; Left ventricular noncompaction; NC; Noncompacted