title: Factors associated with severe aortic dilation in patients with Fontan palliation.
abstract: To describe the range of aortic dimensions and severity of aortic valve regurgitation (AR) in patients after the Fontan operation, examine rate of growth over time and identify risk factors for severe aortic dilation.
  
  
  
  This was a single-centre retrospective study of Fontan patients who underwent magnetic resonance angiography.
  
  
  
  Between 2005 and 2014, there were 235 patients (median age 18.8 years). The aortic root (median Z-score 3.2) and ascending aorta (AAo) (median Z-score 4.1) were dilated. Severe aortic root or AAo dilation (Z-score ≥6.0) was present in 8.5% and 22%, respectively. Multivariable analysis identified older age at Fontan (adjusted odds ratio (AOR): 1.1 per year; 95% CI 1.04 to 1.2; p=0.002), male gender (AOR: 16.3; 95% CI 1.8 to 144.9; p=0.01) and higher mean blood pressure (AOR: 1.5 per 10 mm Hg; 95% CI 1.01 to 2.3; p=0.05) as factors associated with severe aortic root dilation. Older age at Fontan (AOR: 1.1 per year; 95% CI 1.02 to 1.1; p=0.01), male gender (AOR: 3.3; 95% CI 1.5 to 7.5; p=0.004) and left ventricular morphology (AOR: 2.6; 95% CI 1.3 to 5.1; p=0.007) were associated with severe AAo dilation. Over a median of 3.3 years, there was no significant increase in aortic dimension. Most patients (96%) had mild or less AR.
  
  
  
  The Fontan aorta is enlarged at the aortic root and AAo. Older age at Fontan, male gender, elevated blood pressure and left ventricular morphology are associated with severe aortic dilation. Although trivial-mild AR is common, significant regurgitation is infrequent.
authors: Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.|The Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
authors: Yuli Y
authors: YY
authors: Kim
authors: Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.|The Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
authors: Rahul H
authors: RH
authors: Rathod
authors: Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.|The Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
authors: Kimberlee
authors: K
authors: Gauvreau
authors: Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.|The Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
authors: Ellen M
authors: EM
authors: Keenan
authors: Department of Surgery, Harvard Medical School, Boston, Massachusetts, USA.|Department of Cardiovascular Surgery, Boston Children's Hospital, Boston, Massachusetts, USA.
authors: Pedro
authors: P
authors: Del Nido
authors: Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.|The Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
authors: Tal
authors: T
authors: Geva
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001011:Aorta / Q000000981:diagnostic imaging / Q000503:physiopathology; D001014:Aortic Aneurysm / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D001021:Aortic Valve / Q000000981:diagnostic imaging / Q000503:physiopathology; D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D001900:Boston; D004108:Dilatation, Pathologic; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D016015:Logistic Models; D018810:Magnetic Resonance Angiography; D008297:Male; D015999:Multivariate Analysis; D016017:Odds Ratio; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D012737:Sex Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult