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Congenital heart disease
Aortic valve surgery in children
  1. Yves d'Udekem
  1. Department of Paediatrics, University of Melbourne, Murdoch Children's Research Institute, Melbourne, Australia
  1. Correspondence to Yves d'Udekem, Department of Cardiac Surgery, Royal Children's Hospital, Flemington Road, Parkville, Melbourne 3052, Victoria, Australia; yves.dudekem{at}

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In the 1980s, the management of aortic valve disease in children was revolutionised by the introduction of balloon valvuloplasty, and in the 1990s by the adoption of the Ross procedure by the surgical community.w1 w2 In the last decade, balloon dilatation of aortic valve disease in children has become standard practice in many centres. During the same period, concerns about the longevity of the Ross procedure have been raised, improved outcomes after aortic valve replacement in the paediatric population have been reported, and some teams have reported their preference for aortic valve repair.1–3 w3–w5 Consequently, there is some confusion for the clinician who has to determine the best treatment option for his paediatric patient with aortic valve disease.

Pathophysiology of congenital aortic valve lesions

Congenital aortic valve disease

The precise mechanisms leading to congenital aortic valve lesions have not yet been fully elucidated. The predominant theory is that the aortic valve arises from the moulding of three endocardial cushions at the time of the septation of the outflow tract. The complete fusion of the adjoining cushions or cusps will lead to bicuspid or unicuspid valves. All degrees of fusion may exist. The area of fusion may remain well individualised, appearing as a well formed raphe, or may involute completely as in the typical aspect of the unicuspid doming valve (figure 1).

Figure 1

Tricuspid (A), bicuspid (B), and unicuspid (C) aortic valves. The highest level of the cusps coaptation occurs in (a) at the level of the mid-height of the sinuses, while the top of the commissures are at the level of the sino-tubular junction. As adjacent portions of the cusps fuse, the commissural support involutes.

Aortic valve disease as a consequence of other congenital lesions

Alterations in the aortic root morphology and concomitant congenital cardiac lesions may adversely affect the function of the aortic valve. The dilatation of the sinotubular junction, a consequence of the loss of elasticity …

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  • Competing interests In compliance with EBAC/EACCME guidelines, all authors participating in Education in Heart have disclosed potential conflicts of interest that might cause a bias in the article. The author has no competing interests.

  • Provenance and peer review Commissioned; internally peer reviewed.