title: Bicuspid aortic valve: differences in the phenotypic continuum affect the repair technique.
abstract: OBJECTIVE
  Bicuspid aortic valve (BAV) may be considered in the context of a phenotypic continuum of the aortic valve configuration, ranging from severe forms of unicuspid valve to rare forms of quadricuspid valve. In this article, we report our results with BAV repair using surgical techniques tailored to the specific features of the BAV phenotypic continuum.
  
  
  METHODS
  Between September 2003 and May 2009, 31 patients with BAV (mean age 49.9+/-17.3 years; five female; 26 male) were prospectively enrolled for aortic valve repair. The surgical strategy was tailored depending on the anatomical and structural characteristic of the BAV continuum: type 1 real bicuspid (five patients), type 2 bicuspid with raphe (24 patients) and type 3 clefted bicuspid (two patients). An echocardiographic analysis was performed preoperatively, postoperatively, at discharge and every 6 months during follow-up. Kaplan-Meier analysis was used to assess the freedom from re-operation.
  
  
  RESULTS
  One patient died in the intensive care unit due to aortic wall rupture. After BAV repair there was an increase of leaflet coaptation length (from 2.0+/-0.5 mm to 8.2+/-1.9 mm, p<0.01) and a decrease of the diameters of the virtual basal ring (from 24.6+/-3.6 mm to 21.5+/-0.71 mm, p<0.01), of the aortic root (from 43.12+/-13.23 mm to 31.0+/-3.2 mm, p<0.01) and of the sino-tubular junction (from 40.1+/-6.95 mm to 33.5+/-9.19 mm, p<0.05). The aortic regurgitation echocardiographic grade > or =II was found preoperatively in 30 patients and postoperatively in one patient (p<0.01). Six patients underwent an associated aortic valve-sparing procedure (three re-implantation and three remodelling). Kaplan-Meier analysis showed a 96.6% freedom from re-operation at 5 years, with a single new repair procedure and a 100% freedom from aortic valve replacement.
  
  
  CONCLUSIONS
  BAV repair provides a feasible therapeutic option in selected patients with good medium-term results. The surgical techniques should be applied after a careful analysis of the BAV considered in the context of the phenotypic continuum.
authors.affiliation: Department of Cardiovascular Surgery, Luigi Sacco University General Hospital, Via G.B. Grassi 74, Milan 20157, Italy. mangini.andrea@hsacco.it
authors.forename: Andrea
authors.initials: A
authors.lastname: Mangini
authors.forename: Massimo
authors.initials: M
authors.lastname: Lemma
authors.forename: Monica
authors.initials: M
authors.lastname: Contino
authors.forename: Matteo
authors.initials: M
authors.lastname: Pettinari
authors.forename: Guido
authors.initials: G
authors.lastname: Gelpi
authors.forename: Carlo
authors.initials: C
authors.lastname: Antona
mesh_terms: D000328:Adult; D000368:Aged; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery; D017548:Echocardiography, Transesophageal; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012086:Reoperation; D016896:Treatment Outcome