title: Mid- to long-term aortic valve-related outcomes after conventional repair for patients with interrupted aortic arch or coarctation of the aorta, combined with ventricular septal defect: the impact of bicuspid aortic valve†.
abstract: OBJECTIVE
  Bicuspid aortic valve (BAV) is a common risk factor for valve-related problems and occurs more frequently in patients with an interrupted aortic arch (IAA) or coarctation of the aorta (CoA), combined with a ventricular septal defect (VSD), than in the general population. We have been using conventional repair for patients with IAA/CoA+VSD, including those with a very small aortic valve (AV). We retrospectively investigated the outcomes of these patients from the perspective of valve morphology.
  
  
  METHODS
  Between 2000 and 2012, 50 consecutive patients underwent conventional repair for CoA/IAA with VSD [one-stage repair, 44 (88%); staged repair, 6 (12%)]. The criteria for conventional repair were as follows: an AV annulus diameter (AVD) z-score of >-6.0; mitral valve annulus diameter z-score of >-3.0; without retrograde flow in the proximal arch. Sixteen (32%) patients had BAV (Group B); the remaining 34 (68%) patients had a tricuspid AV (Group T). The surgical outcomes in both groups were investigated.
  
  
  RESULTS
  No mortality occurred in the cohort. The median follow-up times were 6 years and 3 months (6 months to 11 years and 8 months) and 6 years and 2 months (4 months to 11 years and 4 months) in Groups B and T, respectively (P > 0.05). The preoperative data (median age at repair, median body weight and median AVD) were comparable in the two groups (P > 0.05). Two patients (4%) underwent reintervention in the aortic arch: 1 patient underwent balloon angioplasty for re-coarctation; the other removal of the interposed graft because of somatic growth. In both groups, the AVD became significantly larger at the 1-year follow-up, approximating the normal value. Three (6%) patients underwent a total of eight valve-related reinterventions (balloon angioplasty, 6; Ross operation, 1; valve replacement, 1). All three had BAV, and the AVD was 3.8-5.6 mm (z-score, -3.4 to -1.6). The 5-year valve-related reintervention-free survival rate was 76% and 100% in Groups B and T, respectively (P < 0.01).
  
  
  CONCLUSIONS
  The long-term outcomes after conventional repair under our criteria were acceptable. BAV was a significant risk factor for valve-related reinterventions after conventional repair for IAA/CoA with VSD.
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan Division of Thoracic and Cardiovascular Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
authors.forename: Ai
authors.initials: A
authors.lastname: Sugimoto
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Noritaka
authors.initials: N
authors.lastname: Ota
authors.affiliation: Department of Pediatrics, Fukui Prefectural Hospital, Fukui, Japan.
authors.forename: Chisato
authors.initials: C
authors.lastname: Miyakoshi
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Masaya
authors.initials: M
authors.lastname: Murata
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Yujiro
authors.initials: Y
authors.lastname: Ide
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Maiko
authors.initials: M
authors.lastname: Tachi
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Ito
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Hironaga
authors.initials: H
authors.lastname: Ogawa
authors.affiliation: Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan ksakamoto@sch.pref.shizuoka.jp.
authors.forename: Kisaburo
authors.initials: K
authors.lastname: Sakamoto
mesh_terms: D001013:Aorta, Thoracic / Q000601:surgery*; D001017:Aortic Coarctation / Q000150:complications / Q000601:surgery*; D001021:Aortic Valve / Q000002:abnormalities* / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D002648:Child; D002675:Child, Preschool; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000601:surgery*; D006349:Heart Valve Diseases / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011183:Postoperative Complications / Q000209:etiology; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Bicuspid aortic valve; Coarctation of the aorta; Conventional repair; Interrupted aortic arch