title: Aortic root disease in tetralogy of Fallot.
abstract: OBJECTIVE
  Progressive aortic root dilatation is a recognized feature of tetralogy of Fallot even in patients following initial reparative surgery. The underlying pathophysiology was initially attributed to altered hemodynamics resulting from longstanding volume overloading and stretching of the aortic root from increased right to left shunting. This review explores the pathophysiology and possible mechanisms for the aortic dilatation, and whether these changes are a reflection of the initial hemodynamic stress or a cellular expression of an unrecognized gene associated with conotruncal defects.
  
  
  RESULTS
  The recent publication of two case reports of aortic aneurysm and dissection in tetralogy of Fallot patients re-emphasized the fact that aortic root dilatation can no longer be regarded as a benign problem in tetralogy of Fallot patients. Findings of intrinsic histological abnormalities in the aortic root and ascending aorta of tetralogy of Fallot patients suggest that intrinsic abnormalities may also play an important causative role.
  
  
  CONCLUSIONS
  A better understanding of the pathophysiology will help to formulate future treatment and management strategies in the subgroup of tetralogy of Fallot patients with progressive aortic dilatation.
authors.affiliation: National Heart Center, Singapore.
authors.forename: Ju L
authors.initials: JL
authors.lastname: Tan
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.forename: Siew Y
authors.initials: SY
authors.lastname: Ho
mesh_terms: D001011:Aorta / Q000473:pathology / Q000503:physiopathology*; D001014:Aortic Aneurysm / Q000209:etiology / Q000503:physiopathology*; D001021:Aortic Valve / Q000473:pathology / Q000503:physiopathology*; D001022:Aortic Valve Insufficiency / Q000209:etiology / Q000503:physiopathology*; D004108:Dilatation, Pathologic; D018450:Disease Progression; D006801:Humans; D013771:Tetralogy of Fallot / Q000503:physiopathology* / Q000601:surgery