Tricuspid atresia with imperforate valve: Angiographic findings and surgical implications in two cases with AV concordance and normally related great arteries

The angiographic and intraoperative findings are described in two children with imperforate tricuspid valve with atrioventricular and ventriculoarterial concordance who underwent a successful Fontan operation. It is important from a surgical point of view to distinguish this rare variant of tricuspid atresia from the more classic form with absent atrioventricular connection. The characteristic feature of hearts with imperforate tricuspid valve is the presence of a normally formed right ventricle which, in the presence of ventriculo-arterial concordance, can be used to support the pulmonary circulation. When the imperforate valve is large, it may be possible to remove it, and restore atrioventricular continuity. Alternatively, an atrioventricular type of Fontan repair using a valved conduit should be considered as the treatment of choice in the presence of a suitable pulmonary arterial tree and pulmonary valve.