ORIGINAL ARTICLE Midterm Results of the Ross Procedure in a Pediatric Population: Bicuspid Aortic Valve Is Not a Contraindication S. El Behery J. Rubay T. Sluysmans B. Absil C. Ovaert Received: 14 April 2008 / Accepted: 8 November 2008 / Published online: 3 January 2009 Ó Springer Science+Business Media, LLC 2008 Abstract Our objective was to analyze retrospectively the short- and midterm results of the Ross operation in children and the impact of bicuspid aortic valve (BAV) disease on outcome. From 1991 to 2003, 41 patients (26 male, 15 female) underwent a Ross procedure. Aortic disease was congenital in all but one. Sixty-six percent had BAV. Mean age at operation was 10.13 ± 5.6 years (range, 0.4–18.3 years). Root replacement technique was performed in all but two (inclusion technique). There were two early deaths. Mean follow-up was 6 ± 3.8 years (range, 0.1–14 years). The autograft (neo-aorta) presented absent, trivial, mild, and moderate regurgitation in 42%, 46%, 10%, and 2%, respectively, at latest follow-up. Root dilation was seen in 64% of the patients (mean Z-score, ?3.53 ± 0.04). Four patients (9%) required allograft replacement, two for endocarditis and two for stenosis. Allograft stenosis (gradient [20 mmHg) was detected in 44% of the remaining patients, without symptoms or the need for reintervention. Estimated freedom from allograft replacement at 5 and 10 years was 97% and 89%. Left ventricular dimensions and function were normal in all patients. No difference was found between patients with BAV and those with tricuspid aortic valves in aortic regurgitation or root dilation. BAV was not identified as a risk factor for root dilation. In conclusion, the Ross operation remains an excellent option for aortic valve replacement in children. BAV is not associated with a worse outcome and is not a risk factor for aortic root dilation at medium-term follow-up. Long-term follow-up is, however, required. Keywords Ross procedure Á Children Á Bicuspid aortic valve The use of an autologous pulmonary valve to replace the aortic valve was first described by Ross in 1967 [22]. The guiding principle in this surgery is to use natural tissue when possible. Pulmonary autograft proved durable and grows in proportion to somatic growth, with a low risk of infection and without the need for anticoagulant therapy [9]. Controversies remain mainly because of a prolonged learning curve, the involvement of two valves, and the probable need for reoperation due to degeneration of homograft or autograft failure. Dilatation of the neo-aortic root leading to progressive aortic regurgitation is also of concern [13, 29]. Bicuspid aortic valve (BAV) is present in 1–2% of the population [1]. Recently the issue arose over whether or not a BAV is a risk factor for adverse outcome after the Ross procedure [7, 8, 11]. BAV may be associated with aortic dilatation, aneurysm formation, and aortic dissection due to focal abnormalities within the aortic media, probably related to a combination of hemodynamic factors and genetic predisposition [2, 8, 12]. Although the histologies of the aortic and the pulmonary media differ in the normal situation [8], the pulmonary artery has the same embryo- logic origin as the aorta and undergoes similar degenerative changes [12]. Autograft failure as a result of neo-aortic root dilation in patients with BAV has been suggested to be a S. El Behery Á T. Sluysmans Á C. Ovaert (&) Department of Paediatric Cardiology, Cliniques Universitaires Saint Luc, Universite´ Catholique de Louvain, 10 Avenue de Hippocrate, Brussels, Belgium e-mail: ovaert@pedi.ucl.ac.be J. Rubay Á B. Absil Department of Cardiothoracic Surgery, Cliniques Universitaires Saint Luc, Universite´ Catholique de Louvain, 10 Avenue de Hippocrate, Brussels, Belgium 123 Pediatr Cardiol (2009) 30:219–224 DOI 10.1007/s00246-008-9335-8