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@PHDTHESIS{Rahatianpur:958692,
      author       = {Rahatianpur, Mehrdad},
      othercontributors = {Rüffer, Andre and Brülls, Christian Simon},
      title        = {{T}he association of bicuspid aortic valve on long-term
                      outcome following one-stage repair of aortic arch
                      obstruction associated with ventricular septal defect},
      school       = {Rheinisch-Westfälische Technische Hochschule Aachen},
      type         = {Dissertation},
      address      = {Aachen},
      publisher    = {RWTH Aachen University},
      reportid     = {RWTH-2023-05385},
      pages        = {1 Online-Ressource : Illustrationen},
      year         = {2023},
      note         = {Veröffentlicht auf dem Publikationsserver der RWTH Aachen
                      University; Dissertation, Rheinisch-Westfälische Technische
                      Hochschule Aachen, 2023},
      abstract     = {Bicuspid Aortic Valve (BAV) is a common congenital
                      malformation of the human heart that is identified by an
                      abnormally large aortic valve cusp with a prominent raphe in
                      an area of cuspal fusion. It may be hereditary or familial
                      and is present in $1\%–2\%$ of the population. BAV is
                      associated with valve-related problems that cause aortic
                      valve stenosis or –regurgitation. In addition, congenital
                      abnormalities of the aortic wall such as Interrupted Aortic
                      Arch (IAA), Coarctation of the Aorta (COA), aortic
                      dissection, and aortic aneurysm are more frequently present
                      in BAV disease. Most patients with BAV are unaware of its
                      presence and are at risk for valvular dysfunction and
                      complications during their lifetime. Therefore, follow-up,
                      particularly monitoring of the diameter and the shape of
                      aorta, is extremely important to prevent unsuspected
                      complications such as aortic dissection. Present study
                      reviewed data from 74 patients with IAA (n = 41) and COA
                      with hypoplastic aortic arch (COA-HyAA) (n = 33).
                      Ventricular septal defect (VSD) was present in all patients
                      who underwent early one-stage correction. Only 20 patients
                      had a BAV. Patients’ median age was 7 ± 29 days (range, 2
                      to 150); median weight was 3.3 ± 0.7 kg (range, 1.5 to
                      6.0), with 21 patients < 3.0 kg. We retrospectively
                      evaluated the association of BAV in early and late
                      contemporary operation outcomes, including reoperation and
                      transcatheter reintervention rates, after one-stage
                      correction for IAA with VSD or for COA-HyAA with VSD from
                      November 1999 to June 2016 from single high-volume center.
                      The early mortality was $1.3\%.$ Median follow-up was 5.7
                      years (IQR: 10.48; range, 0.02 to 15.9). There was no
                      mortality during the follow-up. All patients had no obvious
                      neurologic impairment in routine examinations at last
                      follow-up. Reinterventions occurred in 36 $(49\%)$ patients.
                      A potential risk factor for reintervention after IAA and COA
                      with VSD repair was BAV (p = 0.019, Chi²(1)=5.457). In
                      addition, multivariable Cox analyses confirm that BAV
                      (HR=0.381, p=.016), and IAA (HR=0.412, p=0.043) were
                      predictors of late reintervention. Particularly neonates
                      with BAV will possibly require reintervention in the future.
                      Regular lifelong cardiac follow-up is recommended.},
      cin          = {533540-4 ; 936810},
      ddc          = {610},
      cid          = {$I:(DE-82)533540-4_20140620$},
      typ          = {PUB:(DE-HGF)11},
      doi          = {10.18154/RWTH-2023-05385},
      url          = {https://publications.rwth-aachen.de/record/958692},
}